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Gen Derm Flashcards

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1
Q

Heerfordt’s syndrome

A

Variant of sarcoid: Uveoparotid syndrome

  • *facial nerve palsy
  • BIL anterior uveitis
  • Parotid gland enlargement
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2
Q

Lofgren’s syndrome

A

Acute sarcoid

  • fever
  • migratory polyarthritis
  • E nodosum
  • BIL hilar adenopathy
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3
Q

Mikulicz syndrome

A

Sicca symptoms in sarcoid!
complex constellation of symptoms seen in sarcoid and other:
-lacrimal and parotid enlargement=sicca symptomes

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4
Q

Parinaud oculoglandular syndrome

A

Variant of sarcoid: ipsilateral conjunctivitis and parotid gland enlargement

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5
Q

Darier-Roussy disease

A

Sarcoid panniculitis

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6
Q

Lupus pernio

A

Variant of sarcoid as papulonodules on the face

-associated with chronic lung disease and bone cysts

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7
Q

sarcoid panniculitis

A

Darier-Roussy disease

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8
Q

The Laminin formerly known as Laminin-332

A

Laminin 5 (Intermediate filaments)

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9
Q

What is MADDPORES?

A

The pneumonic for EPS! (Elastosis Perforans Serpiginosa)

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10
Q

Name all of the diseases that EPS is found in?

A 
MADDPORES!
Marfans
Acrogeria
Downs
D-penacillamine
Psuedoxanthoma elasticum
Osteogenesis Imperfecta
Rothmund Thompson
Ehlers Danlos 
Scleroderma
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11
Q

What medicine causes EPS?

A

D-penacillamine

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12
Q

A person with Wilson disease got a weird serpiginous thing on their neck, what is this?

A

EPS - this is because D-penacillamine is an increased risk factor for developing EPS

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13
Q

Which genoderm carries an increased risk of developing osteosarcoma?

A

Rothmund Thompson (1/3 of patients get it!) and nothing else does this

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14
Q

p53 is located on which chromasome?

A

the long arm of chromasome 17

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15
Q

the majority of p53 mutations involve which part of the gene?

A

the sequence specific DNA binding domain

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16
Q

Underphosphorylated Rb binds to which transcription factor to prevent the cell from entering S phase?

A

E2F

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17
Q

a connective tissue nevus in Tuberous Sclerosis

a connective tissue nevus is Buschke-Ollendorff syndrome

A

Shagreen Patch

dermatofibrosis leticularis disseminata

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18
Q

Name two common allergans in topical steroid vehicles?

A
  1. propolyne glycol

2. sorbitan sesquidate

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19
Q

Name two common allergans in topical steroid vehicles?

A
  1. propolyne glycol

2. sorbitan sesquidate

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20
Q

How many CMEs do you need a year?

A

50

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21
Q

What lung finding in Birt Hogg Dube

A

spontaneous pneumothorax

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22
Q

carpal ankylosis is pathoneumonic for which syndrome?

A

Adult onset Stills disease (carpal ankylosis is the fusing of the two bones)

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23
Q

generalized pustular psoriasis in pregnancy is also referred to as:

A

Impetigo herpetiformis

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24
Q

Acrokeratosis Verruciformis

A

Darier’s Disease (ATP2A)

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25
B1
Thiamine
26
B2
Riboflavin
27
B3
Niacin
28
B5
patothenic acid
29
B6
Pyridoxine
30
B7
Biotin
31
B9
Folic Acid
32
B12
Cobalamin
33
Thiamine
B1
34
Riboflavin
B2
35
Niacin
B3
36
Pantothenic Acid
B5
37
Pyridoxine
B6
38
Folic Acid
B9
39
Cobalamin
B12
40
Oro-ocular genital syndrome
deficiencies in B2 (riboflavin) and B6 (pyridoxine)
41
Pellagra
Vitamin B3 deficiency (Niacin) ``` Diarrhea Dermatitis Dementia "casal's" necklace pigmentation of the dorsal hands and feet ```
42
Beri Beri
Thiamine (B1) deficiency
43
Sideroblastic anemia
Vitamin B6 (Pyridoxine)Deficiency
44
The genetic form of zinc deficiency
Acrodermatitis Enteropathica
45
The genetic form of biotin deficiency
Holocarboxylase Deficiency
46
Megaloblastic anemia
Folic acid (Vitamin B9) deficiency OR Cobalamin (Vitamin B12) deficiency
47
"macrocytosis with hypersegmented neutrophils"
pathomnemonic for Folic acid (vitamin b9) deficiency
48
Which drugs cause PML?
Cyclosporine Mycophenolate mofetil natalizumab rituximab
49
why should people with first degree relatives who have MS not take TNF-alpha inhibitors?
TNF inhibitors have increased risk for demyelinating neuropathies
50
dapsone and thalidomide - which type of neuropathies are associated
dapsone - motor neuropathies (reversible) | thalidomide - sensory neuropathies (irreversible)
51
Dry Beri Beri
peripheral neuropathy | found in thiamin deficiency
52
Wet beri beri
congestive heart failure found in thiamine deficiency
53
what vitamin deficiency can cause a peripheral neuropathy?
thiamine B1 *dry beri beri
54
Glossitis | peripheral neuropathy
Dry beri beri - thiamine deficiency
55
polish rice diet
thiamine deficiency
56
coenzyme in carbohydrate metabolism
thiamine (b1)
57
vitamin in collagen formation
vitamin c
58
vitamin that plays a role in the formation of glucose
thiamine
59
oral ocular genital syndrome
Vitamin B2 Riboflavin
60
``` magenta-colored tongue angular chelitis seb derm genital dermatitis photophobia ```
Oral ocular genital syndrome (Vitamin B2 riboflavin deficiency)
61
deficiency found in celiac sprue
vitamin B2 riboflavin
62
vitamin responsible for production of gluathione
Vitamin B2 riboflavin
63
Vitamin responsible for pellagra
Vitamin B3 Niacin
64
``` photosensitive eruption on the face neck and upper chest diarrhea angular chelitis perianal dermatitis disorientation ```
Vitamin B3 Niacin This is Pellagra!
65
Casals necklace
pellagra - vitamin B3 Niacin
66
vitamin causing a peripheral neuropathy
thiamine *vit B1 | pyridoxine *vit B6
67
Vitamin that is a tryptophan precursor amino acid
Niacin *vit b3
68
deficiency associated with a diet composed of entirely corn
Niacin *vit B3
69
deficiency caused by a use of isoniazid (INH)
Niacin *vit B3 AND Pyridoxine *vit B6
70
vitamin deficiency caused by carcinoid syndrome
niacin *vitamin b3
71
Vitamin def associated with cirrosis and uremia
Vitamin B6 Pyridoxine
72
UVB + what = Vitamin D?
7-dehydrocholesterol
73
foamy white spots on the conjunctiva
Bitots spots (vitamin A excess)
74
What skin findings to pts with Vitamin D deficiency have?
alopecia (NO skin rashes!)
75
lab abnormality in decreased Vitamin K
prolonged PT
76
active form of vitamin D
calcitriol
77
what kind of fatty acids can the body not synthesize?
unsaturated FA
78
spoon shaped nails due to iron deficiency
koilonychia
79
Patients on Targretin should not use which common household product?
DEET! For some reason Bexarotine (targretin) combines with DEET to increase DEET toxicity *Alitretinoin also causes this side effect
80
A patient treated got a topical "beauty cream" from a doctor to treat his psoriasis, and after working in the yard in the summer he gets sweaty diaphoretic, ect. What happened?
DEET toxicity. The psoriasis med was likely targretin (bexarotine, binds RXR receptors), and it interacts with DEET to cause DEET toxicity
81
Which topical retinoid is applied 3-4 times per day to treat CTCL?
Targretin (bexarotene)
82
Which topical retinoid is used to treat Kaposi's sarcoma?
Ali-tretinoin (9-cis trans retinoic acid) it is one of the naturally derived
83
another name for alitretinoin
9-cis-retinoic acid
84
Which two topical retinoids are the only ones which bind to the RXR receptor?
Bexarotene (Targretin) -RXR exclusively | Alitretinoing (9-cis-retinoic acid) RXR and RAR
85
Which two topical retinoids are category X?
targretin (bexarotene) and tazorac (tazarotene)
86
Which key cellular component of the development of acne is blocked by topical retinoids?
TLR-2 (which interacts with p acnes to form the microcomedome, the precursor lesion to inflammatory papules and comedomes)
87
Granular cell tumor is derived from what tissue?
Neural tissue
88
Melanocytes appear in which week of gestation?
10 weeks
89
Which two cutaneous B cell lymphomas have a good prognosis?
Follicle center, and marginal zone
90
Which B cell lymphoma is most closely related to Borriela infection?
Marginal zone lymphoma
91
BCL-2 FOX-1 MUM are all markers for which skin disease?
Primary cutaneous diffuse large B cell lymphoma, leg type
92
Marker that is positive in almost all of the Follicle center B cell lymphomas
BCL-6 also CD10
93
what is the wavelength used in NB-UVB therapy?
311nm
94
which cutaneous B cell lymphoma is associated with Borriella?
Marginal
95
Most common cosmetic preservative to cause ACD?
Quaternium-15
96
contact allergen found in vaccines
thimerosal
97
What do you patch test for if trying to find an allergy to hydrocortisone?
Tixocortol-21-pivalate
98
What do you patch test for if you want to find an allergy to triamcinolone?
Budesonide
99
What do you patch test for if you want to find an allergy to fluocinonide?
Budesonide
100
What do you patch test for if you want to find an allergy to clobetasol or betamethasone?
Hydrocortisone-17-butyrate
101
Dont eat which two foods if you have a bad allergy to poison ivy?
Cashews or mangos
102
What is Baboon syndrome?
An ACD on the buttocks caused by ingestion of some sort of pesto pasta containing cashews in a person who is allergic to poison ivy
103
What family is poison ivy a part of?
Anacardiacea
104
Wavelenth of narrowband UVB
311-313
105
PUVA therapy peaks at what wavelength:
352
106
wavelength 290-320 is what?
UVB spectrum
107
Excimer laser is what wavelength?
308
108
UVA2 spectrum is what wavelengths?
320-340
109
UVA1 wavelengths
340-400
110
what wavelengths do you do PDT at?
400-700 (blue and red light)
111
contraindication for phototherapy
thiazides, doxycycline, and lupus
112
most common DNA photoproduct:
cyclobutane-pyrimide dimers (CPD)
113
most frequent cyclobutane-pyrimide dimer:
T-T
114
signature DNA mutation related to UVB
C-->T (cyctoside changed to thymine)
115
what is the mechanism of action of mutations from: UVA UVB
UVA- from ROS | UVB -from formation of dimers
116
pts with pemphigoid gestationis have an increased risk of what post partum?
graves disease
117
why do first generation antihistimines make you drowsy?
because they are LIPOPHILIC - and thus can cross the blood brain barrier
118
How can first gen antihistamines cross the blood brain barrier?
they are LIPOPHILIC
119
Fexofinadine trade name, and prodrug name
Allegra, its prodrug is 1st gen terfinadine
120
which second gen antihistamine is not proven safe to use in pregnancy?
fexofenadine (Allegra)
121
which second gen H1 blocker is metabolized by CYP3A4 inhibitor and why does that matter?
Loratidine (Claritin) - because this is one of the major inhibitors in the liver where it is metabolized and theoretically could interact with another drug like a macrolide or grapefruit juice to increase blood levels of loratidine = cardiac arythmias
122
which second gen H1 blocker is the metabolite of a first gen H1 blocker (and which one is it?)
zyrtec (certirizine) is the metabolite of hydroxizine (Atarax)
123
which antihistamine do you take in pregnancy?
chlorpheniramine!
124
Gene mutated in both atopic derm and ichtyosis vulgaris
Fillagrin
125
what are dennie morgan lines?
the two lines below the canthus in atopic derm patients
126
Contact urticaria is what kind of hypersensitivity?
Type I
127
what is the most common underlying malignancy seen in someone with erythema gyratum repens?
lung cancer
128
Bacterial phage that causes bullous impetigo
Phage II, type 71
129
bacteria causing impetigo causing bullous impetigo
impetigo = staph >strep bullous impetigo= ONLY staph
130
what are pastias lines?
seen in scarlett fever: they are dark lines of confluent purpura found in the axillary and antecubial folds
131
sharply bordered, punched out ulcer with yellow leathery psudomembrane
cutaneous diptheria
132
difference between erysipelas and erysipeloid
erysipelas = strep infection erysipeloid= erysipelothrix, fish handlers disease (also a gram positive bacteria tho)
133
Madura foot with red grains is caused by what bacteria?
Actinomadura
134
Actinomycetoma (Madura foot) with white grains caused by what bacteria?
Nocardia
135
Madura foot caused by yellow grains
Streptomyces
136
PAPA syndrome
pyogenic arthritis PG acne
137
SAPHO syndrome
``` Sweet's with: synovitis acne pustules hyperostosis osteitis ```
138
synonym for Intravascular Papillary Endothelial Hyperplasia
Massons tumor
139
Prodrome of fever and backache - macules/papules initially on face, spreads to trunk and extremities - papules then turn to vessicles with central umbilication * *lesions are all the same age
-Variola (smallpox)
140
Variola virus causes:
Smallpox
141
Name the findings in POEMS syndrome
``` Polyneuropathy Organomegaly Endocrine abnormalities Monoclonal gammopathy Skin findings (glomeroloid hemangioma) ```
142
What vascular neoplasm do you see in POEMS syndrome?
Glomeruloid Hemangioma
143
In what two syndromes do you find a Glomeruloid Hemangioma?
POEMS and Castlemans syndrome
144
Angiokeratoma corporis diffusum is found most famously in what disease?
XLR Fabry disease
145
Multiple clustered angiokeratomas in a bathing suit distribution
Angiokeratomas Corporis Diffusum
146
Multiple angiokeratomas on the fingers and toes, associated with chillblains and acrocyanosis
Angiokeratosis of Mibelli
147
A group of little papules that look like cherry angiomas, usually present from birth, on the arms and legs
Angioma circumsciptum
148
Is TH1 or TH2 seen in chronic atopic derm?
TH1 for chronic atopic derm TH2 for acute atopic derm
149
Is TH1 or TH2 seen in chronic atopic derm?
TH1 for chronic atopic derm TH2 for acute atopic derm
150
Mutations in FILLAGRIN (epidermal cornification protein) are seen in what two disorders?
Atopic Dermatitis & Icthiosis Vulgaris
151
Hertoge sign
Absense or thinning of the lateral eyebrows in atopic derm
152
Which antihistimine has T cell suppressor therapy?
Cimetidine
153
keratins found in suprabasal layer
K1/K10 AND highly expressed K9 in suprabasal layer of palmoplantar epidermis
154
what site is K9 highly expressed in?
Suprabasal layer of palmoplantar epidermis
155
BASAL layer keratins
K5/14
156
palms and soles keratinocytes
K6/17
157
Name two molecules in the cadherin family
Desmoglein | Desmocollin
158
Mutation in steatocystoma multiplex
Keratin 17
159
Steatocystoma multiplex can be seen in what genoderm?
Pachonychia congenita Type II | mutation in Keratin 6a, 17
160
What is the mutation in Pachonychia congenita Type II?
Keratin 6a, 17
161
What skin finding is associated with Pachyonychia Congenita due to mutation in Keratin 17?
Steatocystoma multiplex
162
What embryonic layer is hair derived from?
Ectoderm With the exception of the dermal papilla which is derived from mesoderm!!!
163
What layer is the dermal papilla derived from?
Mesoderm
164
What is the widest diameter of the hair follicle called?
Critical line of auber
165
Which part of the hair segment does the bulk of mitotic activity occur?
The inferior segment right below the critical line of auber
166
Which part of the hair do the melanocytes that determine hair color live?
The inferior segment (in the bulb)
167
Name the layers of the hair from the outer-most to the inner most
``` Glassy membrane Outer root sheath Henle (IRS) Huxley (IRS) Cuticle (IRS) Hair shaft cuticle Cortex Medulla ```
168
Where in the hair do you find the outer root sheath?
The entire hair follicle! It kertatinizes in the infundibular portion and has tricholemmal characterization in the isthmus and the inferior segment
169
Where is the inner root sheath found in the hair follicle?
Found ONLY in the lower segment (not found in the isthmus or the infundibulum)
170
Which layer of the hair follicle contains the majority of the hair KERATINS
The corseted
171
Which layer of the hair follicle maintains the integrity of the hair fibers?
The cuticle layer
172
What is the lower portion of the hair follicle?
Extends from the bulge of the isthmus (insertion point of arrest or pili) down to the bulb of the follicle
173
What portion of the hair follicle contains stem cells?
The bulge
174
Where is the insertion point for the arrector pili muscle?
THE BULGE
175
What term in the hair follicle is used to describe a collection of mesenchymal cells which protrudes into the hair bulb?
The dermal papilla
176
Which hair cycle is the growth phase?
Anagen
177
How many hairs are in anagen at one time?
85%
178
Which hair loss syndrome has miniaturization of telogen and catagen follicles?
Alopecia areata
179
On dermascopy, what would you see in AA?
"Exclamation point hairs"
180
Alopecia with varying lengths of broken hair
Trichotillomania
181
Deformed hair shafts
Trichomalacia
182
Which hair disorder do you find trichomalacia?
Trichotillomania
183
In which type of alopecia would you find an increased number of telogen hairs, normal number of follicles, increased vellous hairs
Androgenetic alopecia
184
Comma hairs, or corkscrew hairs
Tinea capitis
185
Bamboo hair
Trichorrhexis invaginata - Netherton syndrome
186
Tufted hair
Multiple hair shafts emergin from 1 follicle - see in scarring alopecia like folliculitis decalvans and dissecting cellulitis
187
Name all of the herpes viruses
``` HHV1- oral herpes HHV2-genital herpes HHV3-VZV(chicken pox, shingles) HHV4-EBV, mono, Gianotti Crostii, Burkitts, Hodgkins HHV5- CMV HHV6-Roseola HHV7-Pityriasis Roseola HHV8- Kaposi's, Castlemans ```
188
Which HHV causes PR?
HHV7
189
Which HHV causes Kaposi
HHV8
190
Which HHV causes Burkitts
HHV4
191
Which HHV causes roseola?
HHV6
192
Which HHV causes VZV
HHV3
193
Which HHV causes Gionotti Crosti?
HHV4
194
Which HHV causes CMV?
HHV 5
195
Causes of EM
HSV Medication induced: NSAIDS, anticonvulsants MYCOPLASMA
196
Difference between EM major and minor
No oral lesions in EM minor
197
Which HLA class is most strongly associated with SCLE?
HLA-B8
198
Botulinum toxin binds to heavy chain or light chain?
Heavy chain
199
The antigen in DH
Transglutaminase
200
What thing do you want to check in a baby with neonatal lupus?
ECG - 50% of these babies will have permanent 3rd degree heart block!
201
What are the chances the next baby of a sibling with neonatal lupus will also get neonatal lupus?
25%
202
Which type of porokeratosis has the highest risk of turning into a squam?
Linear porokeratosis
203
Trichoeps Cylindromas Spiradenomas BCCs
Brooke Speigler
204
Brooke Speigler
Trichoeps Cylindromas Spiradenomas BCCs
205
Brooke-Speigler gene defect
CLYD (cylindromatosis)
206
Classic triad: alopecia, diaper dermatitis, diarrhea
Acrodermatitis enteropathica (zinc deficiency)
207
Gene for acral melanoma
C-KIT
208
Mucosal melanoma
C-kit
209
Uveal/Ocular melanoma
GNAQ | Highly aggressive metastatic ocular is BAP1
210
Non sun damaged skin/intermittent high intensity sun exposed melanoma
BRAF
211
Lentigo maligna gene
C-KIT
212
Gene defect in chronic sun exposed skin melanoma
C-KIT
213
Metastatic ocular melanoma gene
BAP-1
214
Familial melanoma gene
CDKN2A, BAP
215
Gene mutation in XP melanoma
PTEN
216
Gene mutation in congenital nevi
NRAS
217
Spitz nevi/spitzoid melanoma gene mutation
HRAS
218
Melanin needs what element to make pigment
Copper
219
What specialty should you refer someone to if they begin making multiple halo nevi after age 40 suddenly?
Optho! May indicate ocular melanoma (GNAQ)
220
What HLA type has the highest risk of developing psoriasis?
Cw6
221
How much of your hair is in anagen phase at once?
95%
222
How much of your hair is in telogen phase at once?
5%
223
Name three drugs associated with gingival hyperplasia
Phenytoin Nifedipine Cyclosporine
224
What type of monoclonal gammopathy is associated with scleromyxedema
Lambda light chain
225
An accessory tragus represents an anomaly of what anatomic structure?
First brachial arch
226
Butchers warts are caused by which HPV type?
HPV 2 and HPV 7
227
How many Diabetes patients have NLD?
3%
228
How many NLD patients have diabetes?
30%
229
HPV type Butcher's warts
2, 7
230
HPV flat warts
3, 10
231
HPV type plain warts
3,10
232
HPV type Hecks disease
13, 32
233
HPV type condyloma acuminata
6, 11
234
HPV type verrucous carcinoma
6,11
235
HPV type EDV
Too many to count! 2,3,5,-8-10, 12, 14
236
HPV type common wart
1,2,4
237
HPV type plantar wart
1
238
HPV type Bowenoid papulosis
16,18
239
What two kinds of proteins does the HPV virus encode?
E (early) and L (later) E6 and E7 L1 and L2
240
What does the HPV E6 and E7 protein encode?
Viral DNA replication, which decreases p53 function (E6) and bind Rb (E7) Vs L (late proteins), which encode viral STRUCTURAL PROTEINS
241
Which HPV proteins does the Gardisil vaccine encode?
L (late proteins) 6, 8, 11, 18
242
PCT has what finding in the urine? How about stool? RBCs?
UD (uroporphyrinogen) - urine Isocopro - stool RBC - normal
243
PCT enzyme defect
Uroporphyrinogen Decarboxylase | Urine dazzles pink
244
Triggers for aquired PCT
``` Hemochromatosis Estrogen HIV HCV Alcohol Polychlorinated hydrocarcbons Iron overload C282Y gene ```
245
What gene mutation is associated with aquired PCT?
C282Y
246
What type of monoclonal gammopathy do you see in scleromyxedema?
IgG - lambda light chain
247
What is the enzyme defect in EPP? (Erythropoietin Protoporphyria)?
Ferrochelatase E(PP) -->F (errochelatase)
248
``` In EPP, what enzymes are found in the: Urine Stool RBC: Plasma ```
Urine: normal Stool: proto RBC: proto Plasma: flouresces
249
Which is the only porphyria to have normal urine findings?
EPP (Ferrochelastase). *Ferra Faucet is a Pro Urine: normal Stool: proto RBC: proto
250
Which porphyria presents with photosensitivity with burning Healing with waxy scars Porphyrin gallstones Hepatic damage
EPP
251
Inheritance pattern of PCT
AD
252
Inheritance pattern of EPP
AD
253
What is another name for Gunthers disease?
CEP (Congenital Erythropoetic Porphyria)
254
Congenital Erythropoetic Porphyria is also called:
Gunthers disease
255
Inheritance pattern for Gunthers disease
AR (Congenital Erythropoetic Porphyria) CEP
256
Enzyme defect in Gunthers disease?
Uroporphyrinogen III cosynthase | This is CEP, Congenital Erythropoetic Porphyria
257
Which porphyria presents with erythrodontia
CEP (congenital erythropoetic porphyria), defect in Uroporphyrinogen III cosynthase (Ur Teeth r Colored*)
258
``` In CEP, name the enzymes found in the: Urine Stool RBCs Plasma ```
``` (CEP enzyme defect is Uro III Cosynthase) Urine: uro Stool:copro RBC: uro Plasma: flouresce ```
259
``` Which porphyria presents with: Extreme photosensitivity Bulla heals with intense mutilated scarring Hypertrichosis Erythrodontia Hemolysis (anemia) Red urine, stains diapers Increased risk of skin CA ```
CEP (Congenital erythropoetic porphyria)
260
Porphyria associated with erythrodontia
CEP
261
Baby has red stains in their diapers
CEP (Congenital Erythropoetic Porphyria)
262
Baby has red teeth and red diapers
CEP
263
Baby has red teeth and red stains in diapers, what enzyme must be deficient?
This must CEP (congenital erythropoetic porphyria), defect in the enzyme Uroporphyrinogen III Cosynthase +++uro found in the urine and blood
264
What type of cancers are kids with CEP at risk for?
Skin cancers
265
Which porphyria carries the most dramatic increased risk of skin cancers?
CEP
266
Which porphyria usually requires a bone marrow transplant?
CEP
267
What wavelengths are absorbed intensely by porphyrins?
400-410 (the Soret band)
268
A child with bulla, sunburns, bad mutilated scars has a splenectomy, which porphyria does this sound like?
CEP (findings would also include red teeth, red diapers, hemolysis)
269
Name the only two AR inherited porphyrias?
CEP & HEP
270
Which porphyria has the same enzyme defect as PCT and what is it?
HEP (Hepatoerythropoietic Porphyria), and the defect is in Uroporphyrinogen Decarboxylase
271
Which porphyria is essentially an overlap between PCT and CEP?
HEP
272
In which porphyria is phlebotomy not effective?
HEP
273
Enzyme defect in AIP (acute intermittent porphyria)
Pophobilinogen deaminase (abdomen is painful, Please Barbituates Doc)
274
``` In AIP, name the findings in the: Urine Stool RBC Plasma ```
``` Acute Intermittent Porphyria: Urine: ALA, PBG Stool: normal RBC: normal Plasma: normal ```
275
Which porphyria has no skin findings?
AIP
276
Which porphyria has neurologic and psychiatric problems along wtih abdominal pain?
AIP - no skin findings!!!
277
What do you find in the urine of someone with psychiatric problems, abdominal pain, and a family history of AD porphyria?
Aminolevulinic acid (ALA) and Porphobilinogen (PBG) This is Acute intermittent Porphyria! (AIP). There are no skin findings, only neuro and psychiatric findings associated with abdominal pain
278
Name some triggers of AIP
``` Drugs (barbituates) Stress Fasting Alcohol Hormonal changes Infections ```
279
How do you treat AIP attacks? (Acute intermittent porphyria)
Remove the trigger, glucose load, and hematin infusion
280
What do you find in the stool of an AIP patient (acute intermittent porphyria)
Nothing! Only find ALA or PBG in the urine, nothing in the stool, RBC, or plasma
281
Name the findings in PAPA syndrome
Pyogenic Arthritis Pyoderma gangrenosum Acne
282
Mutation in PAPA syndrome
CD2BP1 (pyrin-interacting protein) like the familial med fever, muckle wells, ect
283
Patient presents with: - Pyogenic Arthritis - Pyoderma gangrenosum - Acne
PAPA syndrome
284
PG Acne Arthritis
PAPA syndrome: Pyogenic Arthritis Pyogenic granuloma Acne
285
``` Patient presents with: Acne Synovitis Palmoplantar pustulosis Hyperostosis Osteitis ```
``` SAPHO syndrome: Synovitis Acne (congoblata) Palmoplantar pustolosis Hyperostosis Osteitis ```
286
Name the findings in SAPHO syndrome
``` Synovitis Acne Palmoplantar pustulosis Hyperostosis Osteitis ```
287
Another name for Chronic recurrent multifocal osteomyelitis
SAPHO syndrome
288
What is the most common location of inflammatory bone changes in SAPHO syndrome?
Sternoclavicular joint
289
Name three systemic syndromes associated with acne:
PAPA syndrome SAPHO syndrome HAIR-AN syndrome
290
What are the findings in HAIR-AN syndrome?
HypernAdrogenism, Insulin resistance, Acanthosis Nigricans | This is listed in Jain as an acne related condition like PAPA/SAPHO
291
Gene mutation in Lichen Sclerosis
ECM-1 (extracellular matrix protein) *also found in Lipoid Proteinosis
292
What derm condition is due to a defect in Extra-cellular Matrix protein?
ECM-1 Lichen sclerosis, Lipoid Proteinosis
293
Enzyme defect in AIP?
PBG (porphobilinogen deaminase) AIP (Abdomen is painful....) BPD (please barbituates D/c) *bc barbituates are a trigger
294
What is the enzyme defect in Varigate Porphyria?
PPO (protoporphyrinogen oxidase) | ViPs get the PPO health insurance?
295
``` In Varigate Porphyria, what are the findings in the: Urine: Stool: RBCs: Plasma: ```
Urine: ALA/PBG Stool: proto RBC: normal Plasma: flouresce
296
What wavelenth does the plasma of a person with Varigate Porphyria flouresce at?
626nm
297
Hereditary Coproporphyria has a defect in what enzyme?
CPO (coproporphyrinogen oxidase)
298
``` In Hereditary Coproporphyria, what are the findings in the: Urine: Stool: RBCs: Plasma: ```
``` HCP: Urine: ALA/PBG Stool: copro RBC: normal Plasma: doesnt flouresce? ```
299
``` In Hepatoerythropoietic Porphyria, what are the findings in the: Urine: Stool: RBCs: Plasma: ```
HEP- Urine: uro Stool: uro RBCs: proto
300
Sarcoid presenting in children
Blau syndrome
301
What is Blau syndrome?
Child sarcoid
302
Which of the periodic fever syndromes do you NOT treat with anakinra?
Familial Mediterranean Fever - treat this one with colchicine instead!
303
What do you treat Familial Mediterranean fever syndrome with?
Colchicine! (You treat the other ones like Muckle Well and TRAPs with anakinra)
304
Child with: extreme photosensitivity Bulla with subsequent mutilated scarring Hypertrichosis
CEP ...also these kids have the erythrodontia and the red urine stains in the diapers
305
Child with erythrodontia
CEP (Congenital Erythropoetic Porphyria)
306
Name the clinical findings of CEP (Congenital Erythropoetic Porphyria)
Extreme sun photosensitivity Bullae with mutilated scars Erythrodontia Red stains of urine in the diaper
307
What blood finding are kids with CEP at risk for?
Hemolysis
308
Kid with bulla and massive scaring and coproporphyrinogen in their stool. What enzyme is defective?
Uroporphyrinogen III cosynthase
309
Photosensitivity with burning Heals with waxy scars Liver enzymes elevated Has to have gallbladder removed
EPP (Erythropoetic Protoporphyria) | -they gell porphyrin gallstones which leads to hepatic damage
310
Porphyrin gallstones
EPP
311
Tense bulla,erosions, milia and scarring on sun-exposed skin Hypertrichosis on temples Iron overload Facial hyperpigmentation
PCT
312
Erbium:YAG laser can cause damage to which part of the eye?
Cornea (because erbium targets water) Lasers that target pigment will damage the retina
313
Purely ocular form of cicatricial pemphigoid
Beta-4 integrin
314
Malignancy associated form of cicatricial pemphigoid
Laminin 5
315
What is the first/most common physical exam finding of cicatricial pemphigoid?
Oral involvement
316
Antibodies to collagen 7 causes what disease?
EBA
317
Defective gene for collagen 7 causes which disease?
Dystrophic EB
318
Drug most likely to cause BP?
Furosemide
319
Most common drug to cause linear IgA
Vancomycin
320
What size is the BPAG2 molecule responsible for linear IgA?
97kD
321
How big is BPAG2 causing BP?
180kD
322
Antibody associated with sclerodermoid changes in DM
Anti-Ku
323
Cardiac and poor prognosis in DM seen with what antibody
Anti-SRP
324
What are the most common cancers associated with DM in: Men & Women:
Men - lung and GI | Women - ovarian
325
HLA-B51
Bechets
326
What HLA type is associated with Bechets
HLA-B51
327
Chronic candidal infections, vitiligo, thyroid problems
APECED (autoimmune polyendocrinopathy, candidiasis, ectodermal dystrophy) Mutation in the AIRE gene
328
Family and genus of poison ivy
Anacardiacea family | Toxicodendron genus
329
Family and genus of poison sumac
Anacardiacea family | Toxicodendron genus
330
UVB spectrum in nm
290-320
331
Mast cells are found in greatest density in what part of the skin?
Papillary dermis
332
Acrokeratosis Paraneoplastica
Basex sign (multiple keratotic papules on the hands and feet as a sign of SCC Of the upper aerodigestive system, like esophageal SCC)
333
Multiple acral keratotic papules on the hands and feet in a patient with SCC of the esophagus
Acrokeratosis Neoplasticism (Bazex sign) *Bazex syndrome is an XLD syndrome with lots of BCC, follicular atrophoderma an d
334
What layer of the epidermis are keratins 5/14 produced in?
Basal layer
335
What keratins are produced in the basal layer?
K5/14
336
What keratins are produced in the spinous layer?
Keratin 1/Keratin 10
337
What two diseases are due to decrease in lamellar granules?
Flegels disease & Harlequin Ichthyosis
338
What is the #1 component of the cornified envelope (stratum corneum)
Loricrin
339
What is the target of light-based acne therapy?
Coproporphyrin III
340
What androgen is produced by the adrenal glands?
DHEA * testosterone is produced by the gonads, and then converted to DHT (most potent androgen) by 5-alpha reductase in the body * DHEA-S is a weak androgen produced by the adrenal glands
341
Where are the androgen receptors located in the pilosebaceous unit?
In the basal layer of the sebaceous gland, and also on the outer root sheath
342
Name a side effect of isotretinoin
Diffuse idiopathic skeletal hyperostosis
343
Diffuse idiopathic skeletal hyperostosis is a rare side effect of what medication?
Isotretinoin
344
Do not take what kind of medications while on accutane
Tetracyclines (Can cause pseud
345
What vitamin excess can cause an acne form eruption?
B12
346
Second most common place to get osteolytic bone lesions in acne fulminans?
Ankle
347
Follicular occlusion triad/tetrad
Acne Dissecting cellulitis of the scalp Pilonidal cyst HS
348
Name a few adrenal androgens
1-hydroxyprogesterone | DHEA-S
349
Lab finding in PCOS
Increased FSH/LH ratio
350
Name the chemical that most commonly causes industrial acne
2,3,7,8 tetrachlorobenzodioxin
351
Most common cause of industrial acne
Insolvable cutting oils
352
Distribution of industrial acne
Malar cheeks, retroauricular region, scrotum
353
Cephalic neonatal pustulosis
Neonatal acne
354
Acne causing a swollen, "wooden" ruddy face
Morbihans disease
355
Mutation in PAPA syndrome
Purine interacting protein - CD2 binding protein 1
356
PAPA syndrome mutation
CD2 binding protein 1
357
CD2 binding protein 1
PAPA syndrome(pyrine binding protein like in Muckle Wells, familial cold urticaria)
358
What infection do you worry about in someone who takes anti-biopics long term (acne-form eruption)
Gram-Negative Folliculitis (centrofacial pustules, perinasal)
359
Most specific Ab in drug induced lupus
Anti-histone Ab
360
Family of plants that causes phytophotodermatitis
Apiaceae (celery, parsley, parsnip, hogweed)
361
What is the most important component of a Baker Gordon peel?
Croton oil (a deep phenol peel)
362
Name an anti microbial peptide that results in the decreased risk of secondary infection in psoriasis
Human beta-defensin 2 (HBD2) | Also skin-derived anti-leukoproteinase SKALP, and secretory leukocyte protease inhibitor SLPI
363
What cell envelope protein is upregulated in psoriasis?
Involucrin
364
Strongest association with early onset psoriasis
Cw6
365
Associated with early onset psoriasis
B13, DR7, B17, B57
366
HLA associated with psoriatic arthritis
B27
367
HLA associated with guttate psoriasis
B13, B17, CW6
368
What is the Wornoff ring
Blanching area around a PSO plaque due to PGE-2
369
Risk factor for pustular psoriasis?
Hypocalcemia
370
Pustular psoriasis associated with what HLA type?
HLA-B27
371
What cytokine is high in the synovium of patients with psoriatic arthritis?
TNF-alpha (Why TNF-A inhibitors work)
372
Von Zumbusch
Generalized pustular psoriasis
373
What's the most common drug that causes PR?
ACE-Inhibitor (Captopril, Lisinopril)
374
What hastens the resolution of PR?
Erythromycin
375
What is the classic type of PRP?
Type I
376
What's the most common type of PRP in children?
Type VI (Type III is the classic but Type VI is the most common)
377
Most common type of arthritis in psoriatic arthritis
Asymmetric oligoarthritis
378
Most common cause of flare of PRP
Phototherapy
379
Pathopneumonic for Reiters syndrome
Circulate balanitis
380
Name two findings in Reiters syndrome
Keratoderma blenorrhagicum | Circulate Balanits
381
What should you avoid if you have a latex allergy?
Avocados
382
Most common allergic reaction to what color tattoo pigment
Red tattoo pigment
383
Most common (pigmented) fixed drug reaction to which drug
Tetracycline
384
Most common non pigmented fixed drug cause
Pseudoephedrine
385
Most common fixed drug in children
Bactrim
386
#1 cause of pityriasis amniacea
Psoriasis
387
What percent of psoriatic arthritis patients have psoriatic arthritis?
30%
388
Does psoriatic arthritis correlate with psoriasis disease severity?
Yes
389
HLA type associated with psoriatic arthritis
HLAB27
390
Collection of neutrophils in the stratum spinosum in psoriasis
Micropustule of Kogoj | Microabcesses of Munro and in the stratum corneum
391
Micropustules of Kogoj
Collections of neutrophils in the stratum spinosum in psoriasis
392
Topical retinoid used for treatment of psoriasis
Tazorac (tazarotene)
393
What type of light therapy is best used in guttate psoriasis flares
Broad-band UVB (vs NVUVB for other types of psoriasis)
394
What is the only systemic retinoid used for psoriasis
Acitretin
395
In which two types of psoriasis is acitretin effective mono therapy
Erythrodermic and pustular psoriasis
396
Name of the sign in psoriasis where you scrape off a scale to find pinpoint bleeding
Auspitz sign
397
Treatment of choice for impetigo herpetiformis (pustular psoriasis in pregnancy)
Early delivery, prednisone
398
Which type of PRP is the only focal type?
Type IV
399
What does type V PRP entail?
Atypical juvenile form - basically PRP with sclerodermoid changes on the hands and feet
400
Most common drug causing PR
ACE-inhibitors
401
Drug will hasten the resolution of PR
Erythromycin
402
New gene defect found in alopecia areata
ULBP3
403
Lab finding in patients with familial alopecia areata
Thrombocytopenia
404
``` #1 symptom of erythroderma AND #1 extracutaenous finding of erythroderma ```
1. Pruritis | 2. Lymphadenopathy
405
Most common cause of erythroderma in healthy patients AND Most common cause of erythroderma in HIV patients
1. Psoriasis | 2. Drug
406
Where does CARP usually start?
Inframammary
407
Treatment for CARP
Minocycline
408
Variant of CARP on the neck with vertically oriented lesions with wrinkling
Psuedoatrophoderma colli
409
Psuedoatrophoderma colli
Variant of CARP on the neck with vertical plaques and wrinkled skin
410
Cytokines and cell types in chronic atopic derm
TH1 cells and IFN-y
411
Cytokines and cell types in acute AD
TH2 cells, and IL-4, IL-5, IL-12, IL-13
412
Mutation associated with early onset atopic derm
Fillagrin
413
Hertoge sign
Loss of lateral eyebrows in atopic derm
414
Ocular findings in atopic dermatitis
Posterior subcapsular cataracts | Keratoconus (elongation of the cornea)
415
Keratoconus
Elongation of the cornea (see in atopic derm)
416
Food allergy is what type of allergic reaction
Type I
417
Atopic dermatitis one week before period
Progesterone dermatitis (occurs with the LUTEAL phase - 1 weeks before period and resolves a few days after menses)
418
Most common cause of ACD worldwide
Nickel
419
Most common cause of ACD in the USA
Poison ivy (nickel is worldwide)
420
ACD is what type of hypersensitivity reaction?
Type IV (delayed type)
421
What is the most common site of an ICD?
The hands Face is #2!
422
UVA spectrum
320-400nm
423
How long after contact with allergic do you get a phytophotodermatitis?
24-72 hours
424
What two things are required to create a phytophotodermatitis?
UVA (320-400nm) and a fucocoumarins
425
Phytophotodermatitis caused by which spectrum of light?
UVA (320-400)
426
What is berloque dermatitis?
Phytophotodermatitis from a cologne containing bergamot oil
427
What chemical causes Beloque dermatitis
Berganot oil (found in men's colognes)
428
What flower is the #1 cause of ACD in florists?
Tulip!
429
What nail product is the most common cause of eyelid dermatitis?
Tosylamide
430
Tosylamide
Nail product that is the most common cause of eyelid dermatitis
431
What fabric finisher is one of the most commons causes of clothing dermatitis?
Formaldehyde
432
What clothing dye is one of the most common causes of clothing dermatitis?
Disperse blue dyes 106 and 124
433
What is EBA most commonly associated with?
Crohn's disease/IBD (most commonly) >multiple myeloma
434
Antigen in EBA
Type VII collagen (major component of anchoring fibrils)
435
Aquired diseases against Type VII collagen
EBA | Bullous Lupus
436
HLA type present in 97% of DH patients
HLA-DQ2
437
Most common autoimmune association with dermatitis herpetiformis (besides celiac)
Hashimoto's thyroiditis> IDDM > pernicious anemia
438
Name three foods gluten is NOT found in
Oats, rice, corn | IS found in wheat rye and barley
439
Antigenic byproduct of gluten
Gliadin
440
Skin antigen in DH, and gut antigen found in dermatitis herpetiformis
Skin: Transglutaminase 3 Gut: Transglutaminase 2 (Side note - Transglutaminase 1 is mutated in Lamellar Ichthyosis, and transglutaminase 1 is found in the granular layer and is the enzyme responsible for the cornified cell envelope)
441
Dermatitis herpetiformis has what kind of infiltrate in the dermal papilae?
NEUTROPHILIC
442
How many patients with DH have SYMPTOMATIC IBD?
20% | But *90% have some form of celiac on biopsy, its just not always active
443
Dermatitis Herpetiformis is almost indistinguishable in real life from what other blistering disorder?
LABD
444
Linear IgA bullous dermatosis in childhood
Chronic Bullous Dermatosis of Childhood
445
What does DH look like on path?
Granular IgA deposits in the dermal papillae on DIF. On H&E will be a neutrophilic infiltrate in the dermal papillae
446
What serologic test has a high sensitivity
Anti-endomysial | You would think it would be anti-gliadin but that one actually give high false positives
447
Treatment of choice for dermatitis herpetiformis
Dapsone | Only treats the skin, has no effect on the GI tract
448
What is second line treatment for Dapsone if it is not tolerated in linear IgA and DH?
Sulfapyridine (good response with less hemolysis risk)
449
What DH treatment controls both skin and GI disease and is the only way to decrease risk of a MALT lymphoma?
Gluten free diet. (Dapsone only treats the skin in DH)
450
DH patients are at risk for what kind of cancer?
MALT lymphoma
451
What substance should you avoid in DH to prevent a flare?
Iodide
452
SJS/TEN can have antibodies against what cell protein?
Desmoplakin I
453
Eosinophilic accesses seen in:
Pemphigus Vegetans
454
Anti-dsDNA, anti-Smith
SLE, Lupus nephritis
455
Anti-dsDNA
SLE, lupus nephritis, *early onset *correlates with disease activity
456
Anti-rRNP
Neuropsychiatric SLE
457
Anti-Ro (SSA) | Anti-La (SSB)
Mild systemic lupus SCLE Neonatal lupus - congenital heart block
458
Anti-histone
Drug induced lupus
459
Anti-KU
Polymyositis
460
Anti-ssDNA
Risk of SLE in DLE
461
What antibody indicates increased risk of SLE in DLE patients
SsDLE
462
99% of patients with neonatal lupus with congenital heart block have this antibody
Anti-Ro
463
75% of SCLE patients have this antibody
Anti-Ro
464
Highly specific antibody for lupus nephritis
DsDNA
465
Antibody indicating an increased risk in DLE patients to develop SLE
SsDNA
466
Two antibodies highly specific for SLE
DsDNA and anti-Smith
467
Antibody highly specific for SCLE
Anti-Ro
468
Antibody found in neuropsychiatric lupus
rRNP
469
Name most common location for venous stasis leg ulcer
Medial supramalleolar region
470
Degos Disease
Malignant atrophic papulosis (form of vascular disorder with red papules that evolve into porcelain white scars on the extremities)
471
Malignant atrophic papulosis
Degos disease
472
Dowling-Degos
Reticulated pigmented anomaly of the flexures
473
What is the initial presenting sign of scleroderma (systemic sclerosis) in >50% of patients?
Pitting edema of the digits. The other 50% is Raynauds
474
Systemic Sclerosis (Scleroderma) antibodies:
Anti-centromere Anti-topoisomerase (Scl-70) Anti-RNA polymerase
475
Which systemic sclerosis/scleroderma antibody is associated with pulmonary HTN?
Anti-centromere (this one is also most closely associated with CREST)
476
Which scleroderma antibody is associated with pulmonary fibrosis?
Anti-Scl (anti-topoisomerase). *this is the one also most closely associated with systemic sclerosis in general
477
Which scleroderma antibody is most closely associated with severe skin involvement and renal crisis?
Anti-RNA polymerase
478
Anti-topoisomerase
Also known as anti-Scl: marker for systemic sclerosis/scleroderma
479
Anti-Fibrillarin
Associated with internal organ involvement for systemic sclerosis
480
Name the findings in CREST syndrome
``` Calcinosis cutis Raynauds Esophageal dysmotility Sclerodactyly Telangiectasias ```
481
Name the antibodies found in Scleroderma
Anti-centromere : CREST, pulmonary hypertension Anti-topoisomerase (anti-RNP): Sscl, pulmonary fibrosis RNA Polymerase: skin, kidneys Fibrillan : a/w internal organ damage
482
What is the nail finding in severe progressive systemic sclerosis
Pterygium inversum unguis (extension of the hyponychium on the undersurface of the nail plate)
483
Most common site of visceral disease in systemic sclerosis (scleroderma)
GI (90%) * associated with significant morbidity but almost no mortality * the most common cause of death in Sscl is pulmonary
484
What medication will decrease the risk of a renal crisis in systemic sclerosis?
ACE-I
485
New biomarker for skin and lung involvement in scleroderma/systemic sclerosis
CXCL4
486
What type of morphea is most common in adults? In children?
Adults: plaque type morphe Children: linear morphea
487
What cell marker do you see in both morphea and scleroderma
TGF-B *stimulates fibroblast production
488
Morphea is associated with what organism in Europe and Japan?
Borriela afzelii, Boriella garinii
489
Which antibodies are a/w linear morphea in children?
Anti-ssDNA (*important bc a/w significant morbidity)
490
Brunstein Perry vs Perry Romberg
Brunstein Perry- cicatricial pemphigoid limited to head and neck with scarring alopecia & w/o occular findings Perry-Romberg- unilateral atrophy of the face d/w morphea
491
Ss-DNA antibody
A/w linear morphea in children & A/w progression of DLE to SLE
492
What anti-body is characteristic of linear morphea in children?
ssDNA (sidenote it is also associated with progression of DLE to SLE)
493
Morphea of the face leading to hemiatrophy
Perry-Romberg
494
Morphea of the scalp
En Coup de Sabre
495
Atrophoderma of Pasini
Sharp "cliff drop" of normal skin (this is a type of morphea)
496
Linear atrophoderma of Moulin
More benign localized variant of atrophoderma of Pasini, a type of morphea
497
Linear form of atrophoderma (type of morphea)
Linear atrophoderma of Moulin
498
What antibodies do all morphea patients lack that scleroderma patients have?
Anti-Scl70 (topo-isomerase I) Anti-centromere *morphea has anti-topoisomerase II! NOT 1
499
Treatment of choice for moderate to severe morphea
MTX
500
High U1RNP titers are seen in what auto-immune disease
Mixed Connective tissue disease MCTD
501
Antibody marker in MCTD
HIGH titers of U1RNP. Also see a speckled pattern ANA
502
U3RNP U1RNP What are they?
U3RNP - same this as anti-fibrillan. Seen in Systemic sclerosis a/w internal organ involvement U1RNP - high titers seen in MCTD
503
Type I keratins - which ones are they? are the acidic or basic? What chromosome are they derived from?
Type I keratins are acidic. K9-28. Chromosome 17
504
Type II keratins- Which are they Are they acidic or basic What chromosome are they derived from?
K1-8 Basic Chromasome 12
505
K1/K10 found in what layer of the skin
Stratum spinulosum (and really anything suprabasilar)
506
K1,K9 found where?
Palmoplantar skin
507
Which keratins are increased in warts in immunosuppressed persons?
K4,13
508
Name ALL the lab findings in Chung-Strauss
Increased IgE Increased MPO (myeloperoxidase antibody -this is actually p-anca) P-Anca
509
What does ANCA stand for? | And what are they antibodies against?
Anti-neutrophil cytoplasmic antibodies | They are auto-antibodies against various lysosomal enzymes
510
Another name for P-ANCA
Anti-myeloperoxidase antibody
511
Another name for C-ANCA
Anti-proteinase 3 (PR-3)
512
Anti-proteinase 3
C-ANCA (Wegeners)
513
Anti-Myeloperoxidase
P-ANCA (Churg Strauss)
514
Small vessel vasculitis with skin nodules, livedo Granulomatous vasculitis of the lungs (asthma,allergic rhinitis) Can affect GI tract, heart - Does this have P-ACNA or C-ANCA?
P-ANCA this is Churg Strauss
515
Systemic vasculitis involving respiratory tract Granulomatous vasculitis involving kidneys Mucosal involvement - oral ulcers, friable gingival Skin ulcers and nodules looks like PG Does this have a P-ANCA or a C-ANCA lab value?
C-ANCA this is Wegeners
516
Which vasculitis can have red friable gingival, oral ulcers, and ulcers on the body which resemble PG? Can also affect kidneys
Wegeners (P-ANCA/Anti-proteinase-3)
517
Which types of cryoglobulinemia are associated with RF?
Types II,III (the mixed cryoglobulinemias)
518
What underlying associations are found in cryoglobulinemia types II,III
HCV, autoimmune disorders
519
What clinical findings do you see with types II, III cryoglobulinemia?
Palpable purpura, glomerulonephritis, peripheral neuropathy
520
Which cryoglobunemias are mixed?
Types II, III
521
What is the difference between II, III type cryoglobulinemia?
Type II - Monoclonal IgM, Polyclonal IgG | Type III- Polyclonal IgM, Polyclonal IgG
522
What treatment worsens the peripheral neuropathy found in cryoglobulinemia Type II/III?
Interferon
523
What treatment should you not give someone who is having peripheral neuropathy associated with a cryoglobulinemia?
Interferon (of note, peripheral neuropathy occurs with types II, III)
524
Which type of cryoglobulinemia do you get a peripheral neuropathy?
Types II, III
525
Which type of cryoglobulinemia is associated with HCV? | How about autoimmune diseases like lupus?
All types II/III
526
What type of antibodies are found in cryoglobulinemia type I?
Monoclonal IgM>IgG | *and remember NO rheumatoid activity
527
In which type of cryoglobulinemia is rheumatoid factor not associated?
Type I
528
What underlying association is found in cryoglobulinemia type I?
Lymphoproliferative disorders
529
Which type of cryoglobulinemia are leukemias associated with?
Type I
530
Which type of cryoglobulinemia is Raynauds, purpura, acrocyanosis, and arterial thrombosis found in?
Type I
531
Name some clinical findings of Type I cryoglobulinemia?
Raynauds, arterial thrombosis, purpura, acrocyanosis
532
What do you treat atrophe Blanche in anti-phospholipid syndrome with?
Anti-malarial
533
What is the most specific antibody found in anti-phospholipid syndrome. How about the most common?
Specific- anti-beta2-glycoproteins | Most common - anti-cardiolipin
534
How long after starting heparin do you see heparin induced thrombocytopenia?
5-10 days
535
What kind of mutation is associated with erythromelalgia in myeloproliferative disorders?
JAK-2
536
Gene defect in paroxysmal nocturnal hemiglobinuria
PIG-A gene, treat with eculizumab
537
Cancer a/w acanthosis nigricans
GI adenocarcinoma (gastric)
538
Cancer a/w aquired angioedema
Lymphoproliferative disease
539
Aquired diamond shaped scale on the legs (aquired ichythiosis) a/w with what cancer
Hodgkins and NHL (same as aquired angioedema)
540
Cicatricial localized loss of hair a/w with what kind of cancer
Metastatic Breast (called alopecia neoplastica)
541
Primary amyloidosis looks like what and is associated with what malignancy
*perioribital pinch purpura | A/w multiple myeloma
542
Bazex sign
Also called acrokeratosis neoplastica *a/w upper aerodigestive tract Squamish Can occur on the ears also
543
What chemical do you test in the urine if you suspect someone has a carcinoid tumor
5-HIAA (hydroxyindoleactetic acid), a serotonin metabolite
544
Flushing Pellagra-like dermatitis Erythema
Carcinoid syndrome
545
Erythema gyratum repens a/w what malignancy
Bronchogenic carcinoma
546
Sudden growth of downy hair in an adult a/w what cancer
Hypertrichosis lanuginosa acquisita *a/w lung and colon (b/c a hairy mans chest covers both)
547
Cancer a/w necrolytic migratory erythema
Pancreatic (alpha-cell tumor)
548
What type of pancreatic cancer is a/w necrolytic migratory erythema
Alpha-cell tumor
549
NXG a/w what type of cancer?
Myeloma, occasionally a paraproteinemia
550
Sign of Leser-Trelat a/w what cancer
* suddern eruption of SKs | * a/w gastric, colon, breast
551
Sweets syndrome a/w what malignancy
AML
552
Tripe palms
* acanthosis palmaris - a/w Lung cancer (if only palms) - but if it also has acanthosis nigricans then it is still gastric cancer
553
Trousseau sign
*migratory thrombophlebitis, a/w pancreatic cancer
554
Sister Mary Joseph Nodule
Nodule in the belly button a/w stomach, bowel cancer
555
Eruptive xanthomas a/w with what condition?
Diabetes
556
What type of scleroderma is related to DM?
Scleroderma of Buschke (Type III scleredema)
557
What is madarosis
Alopecia of the lateral 1/3 of eyebrows (seen in Hypothyroidism)
558
What is necrolytic acral erythema seen in?
Hep C
559
Hep C patient has hyperpigmented hyperkeratotic lesions on the hands and feet, what is this?
Necrolytic acral erythema
560
Nephrogenic systemic fibrosis a/w what condition?
ESRD
561
NSF is seen in ESRD patients with exposure to what chemical?
Gadolinium based contrast material
562
Who do you not want to give gadolinium based contrast material to? And why?
ESRD patients, bc it will cause NSF
563
Glossitis and pinch purpura see in what
Amyloidosis
564
A patient on chronic hemodialysis gets carpal tunnel and back pain - what should you suspect
Hemodialysis associated amyloidosis *deposition of beta-2-microglobulin into synovial membranes
565
Protein in hemodialysis associated amyloidosis
Beta-2-microglobulin
566
Beta-2-microglobulin
Type of amyloid found in hemodialysis associated amyloidosis
567
Amyloid causing cardiomyopathy, inherited type and aquired
ATTR/TTR (transthyretin) which is a protein that transports thyroxine and retinol
568
Protein see in familial amyloidosis
TTR
569
Melkerson-Rosenthal syndrome
Oreo-facial granulomatosis | Triad of facial nerve palsy, fissured tongue, granulomatous chelitis (swelling of gums and lips)
570
Eosinophilic Granuloma
Localized benign form of Langerhans Cell Histtocytosis
571
Feature of a poor prognosis in LCH?
BRAF V600E
572
BRAF V600E
Indicates a poor prognosis in LCH
573
What common progenitor cell do all the histiocytosis disorders share?
CD34+
574
First line treatment for Staph Scalded Skin Syndrome
Clindamycin
575
Basal ganglia calfications
Cockayne Syndrome
576
Most common cause of drug induced dermatomyositis
Hydroxyurea (can also be caused by statins)
577
Mechanics hands
Strongly associated with anti-syntheses syndrome (form of dermatomyositis)
578
Palmar papules in dermatomyositis associated with what antibody?
Anti-CADM-140 *cadbury eggs in the palms
579
What is the PRP Dermatomyositis overlap syndrome called?
Wong-type dermatomyositis
580
Juvenile Dermatomyositis associated with severe vasculitis is called
``` Bankers variant juvenile dermatomyositis -cutaneous ulceration Muscle infarction GI perforation Widespread calcinosis Severe course with poor prognosis ``` . *hemorrhaging money
581
Child with juvenille DM gets rapid cutaneous ulceration, muscle infarction, perforating bowel, widespread calcinosis, what is this called?
Banker's variate- juvenille DM
582
What finding in DM is actually a good prognostic factor?
Raynauds
583
``` Raynauds Mechanics Hands Interstitial lung disease Non-erosive arthritis Anti-synthetase antibodies Dermatomyositis ```
Anti-synthetase syndrome! *boards favorite
584
Name the findings in anti-synthetase syndrome
``` DM Raynauds ILD Mechanics hands Non-erosive arthritis Anti-synthetase antibodies ```
585
What is a clinical difference between drug induced hydroxyurea caused Dermatomyositis and non-hydroxyurea induced?
Hydroxyurea induced DM NEVER has muscle involvement(and it can start really late - like 60 months after initiation) and non-hydroxyurea ALWAYS has muscle involvement (starts 2 months after the drug)
586
Most common cause of death in dermatomyositis?
Malignancy
587
In DM, which usually occurs first - skin or muscle findings?
Skin findings usually precede muscle findings by 2-3 months
588
Cicatricial pemphigoid with scalp involvement, scarring alopecia, and no mucosal involvement
Brunsting-Perry Variant
589
Brunsting-Perry
Variant of cicatricial pemphigoid with no mucosal involvement, but head and neck involvement with scarring alopecia
590
First line treatment for eosinophilic pustular folliculitis (Ofujis)
Indomethacin
591
Leading infectious cause of deafness
CMV infection of the newborn
592
Blueberry muffin baby Leading cause of deafness Owl eye nucleous
CMV
593
What can be the presentation of CMV in HIV patients?
Lower extremity ulceration GI erosions Perianal erosion Chorioretinitis
594
Complication of Roseola
Febrile seizures
595
Castlemans is caused by what virus?
HHV-8
596
What virus is primary effusion lymphoma associated with
HHV8 (same as Castlemans, Kaposis)
597
What structure in the body (skin) is the target of HPV?
Basal layer
598
What cryo temperature do you need to get to destroy: BCC/SCC Wart Melanocytes
BCC/SCC: -50 Wart: -20 Melanocytes: -5
599
People with EDV have malignant transformation of warts to SCC in what percentage of patients?
50%
600
HPV types Hecks disease
13, 32
601
HPV types 13, 32 are what disease?
Hecks Disease
602
In EDV, what is the most common HPV types that will progress into SCC?
5,8
603
Langerhans cell stains and markers
S100+, CD1A+, Langerin (CD207) *CD207 is the most specific, stains Birbeck granules
604
50% of NXG patients have what kind of complication
Opthalmic
605
Send all NXG patients to what specialty
Optho (>50% of them get ocular complications) & HemeOnc (>80% have an IgG monoclonal gammopathy)
606
>80% of NXG patients have what finding?
IgG monoclonal gammopathy
607
What type of monoclonal gammopathy do 80% of NXG patients get?
IgG
608
Location of multicentric reticulohistiocytosis
Head, dorsal hands, periungal "coral bead" appearance (papules along periungal region)
609
What other organ systems are affected in multicentric reticulohistiocystosis
Mutilating arthritis Nasopharyngeal involvement Solid organ malignancy in >30%
610
Solid organ malignancy found in what percentage of multicentric reticulohistiocytosis?
30%
611
Most common skin site for Rosai-Dorfman disease?
Eyelid>malar cheek
612
S100 is negative in all non-langerhans cell histiocytosis except which two diseases?
Rosai-Dorfman & Indeterminate cell histiocytosis (ICH)
613
What clinical findings make up the xanthoma disseminatum triad?
Cutaneous xanthomas Mucosal xanthomas Diabetes insipidus
614
What lab findings are found in xanthoma dissiminatum
Normolipemic Monoclonal gammopathy Plasma cell Dysplasia
615
Monoclonal gammopathy associated with plane xanthoma
IgG
616
Monoclonal gammopathy associated with Sweets
IgA
617
Monoclonal gammopathy associated with Primary amyloidosis (AL)
IgG
618
Monoclonal gammopathy associated with NXG
IgG kappa
619
Monoclonal gammopathy a/w Scleredema
IgG kappa
620
Monoclonal gammopathy a/w Scleromyxedema
IgG Lambda
621
Monoclonal gammopathy a/w Pyoderma gangrenosum
IgA
622
Monoclonal gammopathy a/w EED
IgA
623
Monoclonal gammopathy a/w POEMS syndrome
IgA, IgG
624
Monoclonal gammopathy a/w Waldenstroms macroglobulinemia
IgM
625
Which familial hyperlipidemias have eruptive xanthomas?
Type I, IV, V. (Types 2-3 have tuberous and tendinous xanthomas)
626
Which familial hyperlipidemias do NOT carry an increased risk for coronary artery disease?
Type I, IV, V (the ones who have increased triglycerides, eruptive xanthomas) *its like the triglycerides seep out into the skin and into the pancreas so they dont have time to mess with the heart
627
Which types of familial hyperlipidemias have a creamy top layer in the serum?
All of the ones with increase triglycerides - so Type I, IV, V
628
Name the defect in familial hyperlipidemia type I
Decrease in lipoproteins lipase (LPL) or Apolipoprotein CII defect
629
Which familial hyperlipidemias have increased risk for pancreatitis
Type I, V (same ones that are associated with eruptive xanthomas)
630
Eruptive xanthomas, DM Obesity Alcoholism
Type IV hyperlipidemia
631
Xanthoma striatum palmare associated with what type of hyperlipidemia
Type III (Apoprotein E)
632
Defect in Type IV hyperlipidemia
Increased production of VLDL
633
Defect in types II, III familial hyperlipidemia
LDL receptor
634
In which familial hyperlipidemias do you see xanthelasma?
Types II, III
635
Hundreds of xanthomas with FLEXURAL predilection, NORMOLIPEMIC, associated with diabetes insipidus
Xanthoma disseminatum (can be associated with monoclonal gammopathy too)
636
Besides hyperlipidemia, what other condition can you see xanthomas associated with?
Primary biliary cirrhosis
637
Triglycerides must be greater than what number for eruptive xanthomas to form?
>3000
638
Tendinous xanthomas are usually found on what structure and are found in which familial hyperlipidemia?
Found on Achilles' tendon | Usually associated with Type II hyperlipidemia
639
Dysbetalipoprotienemia is also known as which familial hyperlipidemia as the pathopnuemonic characteristic is what finding?
Type III familial hyperlipidemia, | Xanthoma striatum palmare
640
What are the preformed ingredients released from mast cells in urticaria?
CHT* having a chat Chymase Tryptase Histamine Heparine
641
Newly formed mediators released from mast cells during urticarial reaction
Protaglandin D2 Leukotriene C4/D4/E4 Platelet activating factor
642
Name the causes of immunologic urticaria
IgE mediated (Type I hypersensitivity reaction) Complement-dependent Immune complex deposition (serum sickness) Autoantibodies (anti-IgE, anti-FceRI) *chronic urticaria
643
What drugs are most often implicated in urticaria
``` Antibiotics NSAIDs Allopurinol Sulfa Opiates Radiocontrast Polymyxin B ```
644
ACE-I (like captopril) causes angioedema through unregulated generation of what substance?
Bradykinin
645
What does CI-INH do?
Prevents spontaneous activation of the complement system (serine protease inhibitor)
646
Which complement factor is low in all types of hereditary and aquired angioedemas?
C4 *C3 is always normal!
647
What are the natures of the levels of C1-INH in type 1 vs type 2 hereditary angioedema?
Type 1= deficient C1-INH | Type 2= normal to high levels of C1-INH, they are just dysfunctional
648
Is C1q elevated, normal, or low in hereditary angioedema?
NORMAL C1q in hereditary angioedema (*it is low in aquired angioedema)
649
``` What would your labs show in hereditary angioedema? C4 C3 C1q C1-INH ```
Decreased C4 Normal C3 Normal C1q Decreased C1-INH
650
What androgens do you treat hereditary angioedema patients with?
Danazol Stanazol
651
Treatments for hereditary angioedema
Anti-androgens including danazol and stanazol Fresh frozen plasma before surgery C1-INH
652
What lab value is decreased in Aquired angioedema that is normal in hereditary angioedema
Decreased C1q
653
C1q is decreased in which: acquired angioedema vs hereditary angioedema?
C1q is decreased in aquired angioedema
654
Which is the only type of angioedema in which the C1-INH level is normal or even increased?
Hereditary angioedema Type II (where the C1-INH is dysfunctional, instead of type 1 where it is absent)
655
Which aquired angioedema is associated with lymphoproliferative disorders?
Aquired angioedema Type I
656
Type I aquired angioedema is associated with what other diseases?
Lymphoproliferative disorders/ B cell lymphoma, multiple myeloma ect
657
Which type of angioedema requires more C1-INH during an attack: aquired or hereditary angioedema?
Aquired angioedema requires quite a bit more C1-INH during an attack
658
Type of tumor associated with necrolytic migratory erythema
Pancreatic tumor (alpha-cell tumor), and if the patient is losing weight and gets DM it is probably a glucagonoma
659
New onset severe intertrigo could be linked to what kind of tumor?
Pancreatic tumor (necrolytic migratory erythema)
660
What percentage of NXG patients have a monoclonal gammopathy, and what is it?
80% | IgG
661
Which conditions have an IgG gammopathy and what type are they?
NXG - IgG kappa | Scleromyxedema - IgG lambda
662
Anti-Ku is what kind of protein?
DNA repair protein (p70/p80 nucleoli protein)
663
DM antibody a/w calicinosis cutis
P140
664
Relapsing polychondritis a/w antibodies against which type of collagen?
Collagen type II
665
#1 cause of mortality in patients with relapsing polychondritis
Pneumonia (due to involvement of the respiratory cartilage
666
Syndrome a/w relapsing polychondritis
MAGIC syndrome (mouth and genital ulcers with inflamed cartilage)= Bechets +relapsing polychondritis
667
What is MAGIC syndrome?
Mouth and genital ulcers with inflamed cartilage I.e. = Bechets + relapsing polychondritis
668
Anti-matrilin-1 (extracellular matrix protein)
Relapsing Polychondritis
669
MAGIC syndrome has antibodies against what type of collagen?
Type II Collagen *bc it is basically Bechets plus relapsing polychondritis (and polychondritis is against type II collagen
670
Systemic sclerosis has increased expression of what type of proteins?
Extracellular matrix proteins (ECM) from dermal fibroblasts
671
Systemic Sclerosis has increased deposition of which type of collagen?
Type III collagen
672
What percent of lichen sclerosis patients will develop into SCC?
5%
673
What other autoimmune diseases is lichen sclerosis associated with?
Pernicious anemia | Thyroid disease
674
Tumor in argentaffin cells
Mid gut tumor, gives you carcinoid syndrome.
675
What do you treat the head and neck flushing of carcinoid syndrome with?
Cyproheptadine
676
What do you look for in the urine of people with carcinoid syndrome?
Increased 5-HIAA levels (hydroxyindoleacetic acid)
677
What drugs worsen AIP?
Barbituates Griseofulvin Sulfa drugs
678
Dont give which antifungal to a patient with AIP?
Griseofulvin
679
Antibodies in antiphospholipid syndrome
Anti-b2-glycoproteins Lupus anticoagulant Anti-cardiolipin
680
Antibodies found in Sneddons syndrome
Same as found in anti-phospholipid syndrome (anti-b2-glycoprotein, anti-cardiolipin) *Snedden syndrome is like the worse form of anti-phospholipid syndrome, where you get multiple ischemic strokes, persistent livedo reticularis, livedo racemosa , labeled hypertension
681
Sneddons syndrome
The worse form of anti-phospholipid syndrome (all the same antibodies) - livedo racemosa - persistent livedo reticularis - labile HTN - recurrent ischemic strokes
682
What syndrome(s) is b2-glycoprotein found in?
Anti-phospholipid syndrome/ Sneddons syndrome
683
What do you treat atrophe Blanche leg lesions with in lupus associated anti-phospholipid syndrome?
Anti-malarial
684
Main presenting symptom in cholesterol emboli/warfarin blue toes syndrome
Livedo reticularis
685
How long after starting heparin do you see HIT (heparin induced thrombocytopenia)?
5-10 days
686
Gene mutation seen in erythromelalgia?
JAK-2
687
Treatment for erythromelalgia associated with JAK-2 mutation
Anagrelide or aspirin
688
Gene defect associated with paroxysmal nocturnal hemiglobinuria
PIG-A gene
689
Treatment for paroxysmal nocturnal hemiglobinuria
Eculizumab
690
Gene defect in TTP/HUS
ADAMTS13
691
Most common cause of death in Degos disease?
GI BLEED
692
NSF (Nephrogenic systemic fibrosis) is associated with what chronic condition?
ESRD, a/w exposure to gadolinium-based contrast
693
Which condition is a/w gadolinium-based contrast in patients with ESRD?
NSF
694
A patient with chronic kidney disease develops new onset hypertension, woody texture to skin, joint contractures, and tightness of the skin on the hands and the lower legs. What could this be?
NSF (nephrogenic systemic fibrosis/nephrogenic systemic sclerosis/nephrogenic fibrosing dermopathy)
695
What type of antibodies can NSF be associated with?
Anti-phospholipid antibodies
696
Anti-matrilin-1 protein
Relapsing polychondritis
697
Cancer a/w pityriasis rotunda
Hepatocellular carcinoma
698
Epidermal nevus could possibly be a mosaic form of what disease?
EHK
699
``` Type of amyloid in: Primary systemic amyloid Secondary systemic amyloid Hemodialysis associated amyloid Familial Amyloid Senile systemic amyloid ```
Primary systemic: AL light chain Secondary: AA (amyloid associated protein, not immuno) Hemodialysis associated: b2-microglobulin Familial amyloid: ATTR (transthyretin) Senile systemic amyloid (transthyretin)
700
Type of amyloid seen in primary systemic amyloidosis
AL (light chain)
701
Type of amyloid see in secondary systemic amyloidosis
AA (amyloid associated). This is the type of amyloid associated with systemic disease, like chronic RA, periodic fever syndromes and things like that
702
Type of amyloid seen in hemodialysis associated amyloid
B2-microglobulin
703
Type of amyloid seen in both familial amyloid and senile amyloid?
ATTR (transthyretin)
704
What is the shoulder pad sign?
Amyloid deposits around the periarticular soft tissue. Seen in primary amyloidosis
705
Why do ACE-I give you autoimmune urticaria?
Because they cause unregulated generation of bradykinin
706
Intertrigenous plane xanthomas are pathognomonic for which familial hyperlipidemia?
Homozygous familial hypercholesterolemia (Type II)
707
For paraneoplastic pemphigus: Path resembles - Target antigen - Common cause of death-
Path resembles - EM Target antigen - plains Common cause of death- BOOP (bronchiolitis obliterans)
708
Common cause of death in paraneoplastic pemphigus
BOOP - bronchiolitis obliterans
709
Which autoimmune disorder is a/w hypergammaglobulinemic purpura of Waldenstrom?
Sjogren S
710
What oral endothelium receptor agonist may prevent new ulcers in systemic sclerosis?
Bosentan
711
EED is associated with what disease?
HIV
712
What treatment worsens peripheral neuropathy in cryoglobulinemia type II/III?
Interferon
713
Which cryoglobulinemias are associated with a peripheral neuropathy?
Type II/III
714
Permanent livedo reticularis a/w systemic arterial thrombi, labile HTN, neuro sxs
Sneddons syndrome
715
Eosinophilic pustular folliculitis (Ofujis) first line treatment:
Indomethacin
716
Magenta colored tongue
Riboflavin deficiency B2. *also get angular chelitis
717
AFX is a more superficial version of what tumor?
MFH (Malignant fibrous histiocytoma)
718
P. Acnes naturally produces what porphyrin?
Coproporphyrin III
719
What is the target of light based acne therapy?
Coprophyrin III p. 78 Jain
720
Where are the androgen receptors located in the epidermis?
1. Basal layer of the sebaceous glands | 2. ORS of the hair follicle
721
Which androgen is produced by which organ? 1. Testosterone 2. DHEAS 3. DHT
1. Testosterone - produced in the gonads 2. DHEAS - weak androgen produced in the adrenal glands 3. DHT - not produced anywhere! Testosterone is converted into DHT by 5-alpha reductase in the actual tissues themselves, like the hair follicles, the seminal vessicles, the skin, the penis * circulating DHT is 1/20th of circulating testosterone
722
How does taking OCPs help PCOS?
It increases SHBG (sex hormone binding globulin), and decreases the amount of free testosterone *fyi spironolactone blocks the androgen receptor
723
Most well known chemical causing industrial acne? (This is found as an irritant in agent orange)
2,3,7,8 tetrachlorobenzodioxin (most common chemical to cause chloracne)
724
Acne found on the malar cheeks, retroauricular, and scrotum
Chloracne
725
Name a common EDGFR that causes acne
Cetuximab
726
Autosomal dominant syndrome in which you get resistant acne and recurrent bone lesions
Apert syndrome
727
What is Apert syndrome?
AD- get bone defects and resistant acne FGFR2
728
In what syndrome do you get pyogenic arthritis, acne, and PG?
PAPA syndrome (pyogenic arthritis, PG, acne) *mutation in CD2BP1 aka PSTPIP1
729
What is another name for Apert syndrome?
Acrocephalosyndactyly
730
Another name for acrecephalosyndactyly
Apert syndrome (resistant acne plus bone lesions)
731
Polymorphisms in which TLR may be protective against acne fulminans?
TLR-4
732
Gene mutation in Apert Syndrome
FGFR2
733
Genoderm wtih rosacea-like eruption and verrucous lesions like SKs
Haber's syndrome
734
What is Haber's syndrome?
Genoderm with rosacea-like eruption and verrucous lesions like SKs
735
What cytokines are downregulated in the treatment of rosacea with azelaic acid?
Kallikrenin 5 *and also Cathelicidin!
736
Kallikrenin5
Cytokine that is downregulated with azelaic acid treatment for rosacea
737
What percentage of patients with rosacea have ocular rosacea?
50%!
738
Name some gram negative organisms that commonly occur in gram negative folliculitis
Enterobacter, Proteus, Klebsiella, E. Coli
739
Treatment of choice for Ofujis disease
Indomethacin *Ofujis is eosinophilic pustular folliculitis, occurs in 30 year old Japanese men
740
What are the characteristics of the nerves that control sweating?
They are anatomically sympathetic but function as cholinergic
741
Freys syndrome is caused by disruption of what nerve?
Auriculotemporal nerve (of V1)
742
What chromosomal region is present in over 50% of Psoriasis patients and contains the cw6-allele? Also what chromasome is it on?
The PSORS1 (psoriasis susecptibility region)! Which is located on chromasome 6p
743
What chromosome is PSORS1 located on?
Chromosome 6p
744
What important gene does the PSORS1 allele contain?
Cw-6
745
Which psoriasis allele is most strongly associated with early onset and guttate psoriasis?
Cw-6
746
What two types of psoriasis is HLA-cw6 assciated with?
1. Early onset 2. Guttate *and actually its found in over 50% of adult onset PSO patients as well
747
HLA B27 is associated with what three type of psoriasis?
Psoriatic arthritis Sarco-ilitis-associated Psoriasis Pustular psoriasis
748
Which allele is most strongly associated with: 1. Pustular psoriasis 2. Guttate psoriasis
1. HLA -B27 | 2. HLA- cw6 (and actually B13/B17 as well)
749
Which cytokine upregulation in psoriasis is responsible for increased keritinocyte proliferation?
STAT-3
750
What two medications induce psoriasis quickly (<4 week period)?
Terbinafine, NSAIDS
751
What two medications induce psoriasis in an intermediate time frame? (4-12 week period?)
ACE inhibitors, antimalarials
752
What two medications induce psoriasis in a long term period? (>12 weeks)
Beta-blockers, lithium
753
What percentage of guttate pso patients progress to plaque type?
40%
754
What is the recommended treatment for impetigo herpetiformis
EARLY DELIVERY
755
What acne syndrome can palmoplantar-plantar pustulosis be associated with?
SAPHO
756
Number 1 cause of pityriasis amiantacea
PSORIASIS
757
What systemic do you use as monotherapy for erythrodermic and pustular psoriasis?
Acitretin
758
Which psoriasis variants do you use Acitretin for?
Pustular Psoriasis | Erythrodermic psoriasis
759
Which psoriasis medication should you not use in combination with salicylic acid?
Vitamin D analogs like Dovonex (calciptriol)
760
Which psoriasis medications should you not use right before NBUVB because it causes tumors?
Vitamin D analogs like dovonex (calcipotriol)
761
Name a couple of risk factors for pustular psoriasis
Hypocalcemia | Pregnancy (impetigo herpetiformis)
762
What lab will be decreased in a patient with pustular psoriasis?
Hypoalbunemia
763
Treatment of choice for pustular psoriasis
Cyclosporine
764
HLA type for pustular psoriasis
HLA-B27
765
Treatment of choice for palmoplantar pustulosis
Acitretin
766
Name a psoriasis medication that commonly causes gingival hyperplasia
Cyclosporine
767
Name some side effects of cyclosporine
``` HTN Nephrotoxicity Gingival hyperplasia HYPERkalemia/hypomagnesemia Gout ```
768
Most common childhood type of PRP
Type IV! (Type III is the type that is most like the adult form, type I, but actually type 4 is the most common in children and just presents with plaques on the hands and knees not generalized)
769
Characteristic of Type V PRP
Sclerodermoid changes
770
Characteristics of Type III PRP
Childhood PRP, similar to adult onset PRP. Not the most come (type IV is for kids)
771
What is the most common location for annular LP?
Penis
772
How long is the offending medication usually taken before drug-induced LP is seen?
Several months
773
Which form of LP carries a slight risk of SCC with longstanding lesions
Erosive or ulcerated LP (found in the oral mucosa and palmoplantar surface) of
774
Nail findings is nail LP
Dorsal pterygium 20+ nail dystrophy Lateral thinning Longitudinal ridging
775
Findings in Graham-Little-Piccardi-Lasseur syndrome
Type of lichoplanopilaris (LPP)= | Typical LP+scarring alopecia of the scalp+ nonscarring loss of axillary/pubic hair
776
Scarring alopecia, type of LP
Lichen planopilaris
777
Specific type of LPP, triad of follicular LP plus scarring alopecia of the scalp and nonscarring alopecia of the genitals
Graham Little Piccardi syndrome
778
What percentage of chronic GVHD patients arise from acute GVHD vs de novo?
It's about 50/50!
779
What is the main cause of death in chronic GVHD?
Infection due to immunosuppression
780
Most common type of solid organ transplant to cause GVHD?
SMALL INTESTINE!!!
781
Single most important predictor of developing GVHD is what?
HLA compatibility
782
Which type of stem cell blood products carry the highest risk for developing GVHD? (Peripheral blood vs cord blood vs bone marrow)
Peripheral blood > bone marrow > cord blood
783
What type of blood treatments for stem cell transplants increase the risk for "delayed onset acute GVHD"
Non-myeloablative regimens
784
What is the most commonly affected organ in all GVHD reactions?
SKIN!
785
What monoclonal antibody is helpful in some cases of chronic GVHD?
Rituximab
786
What are two clinical findings which are very characteristic of acute GVHD?
Acral erythema | Violaceous hue on the ear
787
What other organs are commonly affected in addition to skin in acute GVHD?
GI tract and liver
788
How is liver severity assessed in acute GVHD?
By degree of bilirubin elevation *extent of GI involvement evaluated by the amount of DIARRHEA
789
What drug has been shown to decrease the incidence of visceral GVHD?
Maraviroc (CCR5 inhibitor) *note that it decreases visceral involvement like GI and liver but NOT anything in the skin
790
What cytokine does Maraviroc inhibit?
Maraviroc is a CCR5 inhibitor. It decreases the incidence of visceral (not skin GVHD)
791
What CCR5 inhibitor decreases the incidence of visceral GVHD?
Maraviroc
792
#1 site for LP
Oral/mucosal
793
What percentage of patients with oral LP will develop skin LP?
Only 10%!
794
Shulmans Syndrome
Eosinophilic Fascitis
795
What types of cells does LP lack?
Eosinophils and parakaratosis! (With the exception of drug induced LP and oral LP
796
Treatment of choice for Erythema Dyschromicum perstans
Clofazamine! (Weird. Usually for treating leprosy)
797
Where does impetigo herpetiformis start usually?
In the groin! - then spreads to flexures
798
Risk to fetus with impetigo herpetiformis
Placental insufficiency Stillbirth Neonatal death A/w hypocalcemia
799
Does impetigo herpetiformis occur again with subsequent pregnancies?
YES and also OCPs
800
What trimester does impetigo herpetiformis usually occur in?
Third trimester
801
Which trimester does prurigo of pregnancy occur in?
First *most of the other dermatosis occur in the third
802
Treatment for Intrahepatic cholestasis of pregnancy? And what does this do?
Oral ursodeoxycholic acid - this decreases serum bile acid levels
803
Risk to fetus with intrahepatic chol. Of pregnancy
Prematurity Stillbirth Fetal distress
804
Risk to fetus in PUPP
NONE!!!
805
Which trimester does PUPP usually show up?
THIRD
806
Treatment for PUPP
Topical steroids
807
Which pregnancy does PUPP occur in usually
The first. Does not usually recur with subsequent pregnancies
808
Risk factor for PUPP
Multiple gestational pregnancies
809
Risk to fetus with pemphigoid gestationis
Prematurity and SGA
810
What non-pregnancy diagnosis can pemphigoid gestationis occur in?
Choriocarcinoma
811
What disease are patients with pemphigoid gestationis at risk for post partum?
Graves
812
Which HLA types are pemphigoid gestationis patients strongly associated with?
HLA DR3-DR4
813
Which pregnancy dermatosis does not usually recur with subsequent pregnancies?
PUPP is the only one.
814
Which pregnancy diagnosis spares the umbilicus and which one involves it?
PUPP spares the umbilicus and pemphigus gestationis involves it
815
Side effect of Bexarotene
Reversible hypothyroidism
816
Which retinoid causes a side effect of reversible hypothyroidism
Bexarotene
817
What sort of thyroid dysfunction is Bexarotene associated with?
Reversible HYPOthyroidism
818
What kind of drug reaction does azathioprine cause?
SCLE-like reaction! It's known for this
819
another name for Von Hebras disease?
EM minor
820
How many weeks prior does herpes labialis usually precede EM?
3 weeks
821
Cases of EM caused by Histoplasma capsulatum usually also have what other concomitant condition happening at the same time?
Erythema nodosum
822
Erythema Nodosum plus EM together - which type of infection should you be suspicious of?
Histoplasma Capsulatum
823
Most common cause of EM major
Mycoplasma >HSV (Hocker)
824
What is a useful clinical tool to distinguish EM from SJS/TEN?
EM will be PAPULAR and elevated, distributed in the head and neck, and then the acral areas VS SJS/TEN will always be more macular
825
HLA type of Asian/East Indian people with high risk for SJS/TEN due to carbamazepine
HLA-B1502 *HLA B3101 is Europeans exposed to carbamazipine HLA-B5801 Han Chinese exposed to allopurinol
826
Which drug taken by patients of East Indian/Asian decent increases their risk for SJS/TEN, and what is their HLA type?
Carbamazepine HLA -B1501 *HLA 3101 is Europeans taking carbamazipine
827
HLA type in Han Chinese exposed to allopurinol with increased risk for SJS/TEN
HLA-B5801'
828
What is the main cellular mediator of apoptosis in SJS/TEN?
Granulyisin
829
Granulysin
The main mediator of apoptosis in SJS/TEN
830
Which cellular mediator in SJS/TEN leads to activation of caspases and then eventually apoptosis?
The binding of Fas/FasL
831
In regards to SJS/TEN, Which anticonvulsant does NOT cross react with other medications in its class?
Valproic acid *Lamotrigine does not cross react with aromatic anticonvulsants
832
T/F you can get respiratory involvement in SJS/TEN?
TRUE - in fact in 25%
833
Where do SJS/TEN lesions typically start on the body?
On the trunk! (Vs EM usually on the face and acral)
834
What is the only vital sign in the SCORTEN criteria (SJS/TEN)
HR ``` SCORTEN Age (>40yrs) Associated malignancy HR BUN Glucose Serum bicarbonate >10% BSA ```
835
What are the only 3 lab values in the SCORTEN criteria?
Serum BUN Serum bicarb Glucose ``` *TAMEBUG Tachycardia Age Malignancy Epidermal loss Bicarb Urea Glucose ```
836
How many points on the SCORTEN criteria give you a >90% mortality rate for SJS/TEN?
5
837
Which point of the SCORTEN criteria is the single most important risk factor for mortality?
Serum BICARB
838
FDA recommends screening for which HLA type before giving abacavir?
HLA-B5701 *HIGH RISK FOR SJS/TEN!!!!
839
HLA-B1507 a/w high risk of what disease?
SJS/TEN after giving ABACAVIR!
840
Which drug carries a high incidence of SJS/TEN in patients who are HLA-B1507 positive?
ABACAVIR!
841
Avoid drugs with long half-lives or short half lives in patients with SJS/TEN?
Avoid drugs with long half lives!!!
842
Most common SJS/TEN complication?
Ocular sequelae
843
Most common cause of death in SJS/TEN
Infections (pseudomonas or s aureus)
844
T/F sulfa antibiotics do not cross-react with non-antibiotic sulfonamides like HCTZ and hypoglycemic agents?
TRUE they do not
845
What is the severe variant of PLEVA called which has skin lesions plus fever, lymphadenopathy, Mucocutaneous, pulmonary, GI involvement?
Febrile ulceronecrotic Mucha-Haberman disease
846
Does PLC resolve with hypopigmentation or hyperpigmentation notoriously?
HYPOpigmentation
847
Both PLEVA and PLC have a strict absence of what type of cell?
Eosinophils!!!!!
848
What type of cell predominates in PLEVA? (That helps distinguish it from other diseases)
CD8 positive lymphocytes *also remember PLEVA has an absence of eos
849
Most common FDE: - overall - nonpigmented - children - penis
- overall: Tetracyclines (although Hocker says sulfa is most common overall not TCN - nonpigmented:pseudoepedrine - children: bactrim - penis: sulfa
850
What body site does naproxen FDE have a predilection for?
The lips!
851
Which FDE causing medication has a predilection for the lips?
Naproxen!
852
What chemical which was previously found in laxatives has now been removed due to common occurrences of fixed drug eruption?
Phenolphthalein
853
#1 body site for fixed drug eruption?
Oral and genital mucosa!
854
Adult linear IgA-bullous dermatosis a/w what drug?
VANCOMYCIN!
855
Blue grey pigment in the cartilage
Alkaptonuria
856
Blue-grey pigmentation on the face, ears/catilage, and on the sclera - name the mutation? And what are the complications
This is alkaptonuria - homogentisic 1,2,-deoxygenase gene (HGO) Lots of arthroscopic surgeries, risk of MI due to aortic/mitral valvulitis
857
Three primary organisms to cause nail
Scydillium Saprophiatsis Candida
858
The ice cube test is positive in which of these cold urticaria Primary cold contact urticaria Secondary cold contact urticaria Reflex cold urticaria Familial cold urticaria
ONLY in primary cold contact urticaria
859
Aquagenic urticaria is more common in what patient population
Cystic fibrosis patients
860
What type of drugs are first line for urticarial vasculitis?
NSAIDS
861
Syndrome of chronic urticaria, fevers, bone pain, arthralgias, increased ESR and an IgM gammopathy
Schnitzler's syndrome. "SchMitzlers syndrome"
862
Name the findings of Schnitzlers syndrome
``` Chronic urticaria Chronic urticaria Fevers Bone pain Elevated ESR IgM gammopathy ``` *treat with anakinra
863
What do you treat Schnitzlers syndrome with?
Anakinra *remember Schnitzlers is chronic urticaria plus bone pain, fevers, IgM gammopathy
864
What type of monoclonal gammopathy do you get in Schnitzlers syndrome?
IgM! "SchMitzlers syndrome"!!!!
865
Hypocomplementemic urticarial vasculitis will have what notably on labs?
Low complement levels, anti-C1q antibody
866
What inflammatory marker is the cause of all angioedema without urticaria (HAE, AAE, and ACE-I induced angioedema)
Excess bradykinin
867
What deficiency must you rule out when evaluating a case of angioedema without urticaria?
C1 esterase inhibitor deficiency
868
What is the screening test of choice for hereditary and aquired angioedema?
C4
869
What is used for regular prophylactic treatment for hereditary angioedema, and what is used during acute attacks?
Danazol or Stanazol for ppx, and C1H during acute attacks
870
Which type of angioedema without urticaria is characterized by increased significant facial swelling?
Type III! (which is aquired angioedema usually due to lymphoproliferative disorders
871
What labs should you check if you suspect angioedema without urticaria
C1H, C1q, C4
872
Erythema marginatum happens in kids who were not treated for what kind of infection?
Group A beta-hemolytic strep (seen in the setting of rheumatic fever)
873
Treatment of choice for EED
Dapsone
874
EED is a/w what kind of monoclonal gammopathy
IgA monoclonal gammopathy
875
Poor man's test for cryoglobulinemia
RF!
876
If you see Raynauds of the ears, what is this a clinical clue for?
CRYOGLOBULINEMIA!
877
What are the two ELISA tests that you use for detecting wegeners/Churg Strauss?
MPO - an ELISA that confirms P-ANCA in Churgg Strauss | PR3 - an ELISA that confirms C-ANCA in Wegeners
878
What part of the vascular complex is the immune deposition located in cutaneous small vessel vasculitis?
The post capillary venules
879
What street drug can cause CSVV
Levamisole-laced cocaine
880
What is a common and okay finding to see on DIF when you biopsy CSVV
80% w/perivascular C3 and IgM
881
What is a key predictor of nephritis in children with HSP?
Abdominal pain
882
Urticarial vasculitis is a/w which systemic disease EED vasculitis is a/w which systemic disease?
Urticarial vasculitis - SLE EED- HIV
883
Which disease has renal involvement and which doesnt generally? Wegeners Vs Churg Strauss
Wegeners does! Not Churg Strauss
884
Which is granulomatous: Wegeners or Churg Strauss?
Wegeners! Not Churg Strauss!!
885
Perinuclear staining vs Granular cytoplasmic staining: | Wegeners vs Churg Strauss?
Perinuclear is Churg Strauss (P-ANCA/MPO) Granular Cytoplasmic is Wegeners (C-ANCA/PR3) *maybe think bc Wegeners causes granulomas? "Granular cytoplasmic"
886
If you do not treat Wegeners, within one year what will they die from???
Renal disease!!! 80% 1 year mortality
887
What clinical sign is pathopneumonic for Wegeners?
Strawberry gums
888
Most common cause of pulmonary renal syndrome
MPA (microscopic polyangitis)
889
Omalizumab (Xolair) can actually induce which vasculitis?
Churg Strauss
890
What drug induced lupus erythematosus with a positive P-ANCA?
Minocycline-induced lupus! *technically also levamisole as well
891
Which ANCA vaculidity creates a Th1 response?
C-ANCA (Wegeners) this is why you get granuloma formation in wegeners bc of the Th1 response
892
Which ANCA vasculidity is completely without granuloma formation?
MPA
893
Two key clinical features of Churg Strauss
Asthma and Eosinophilia
894
What is usually the first clinical presentation of Churg Strauss?
Adult onset asthma Nasal polyps Allergic rhinitis *then second stage later on comes eosinophilia and then the vasculitis in the third stage
895
Name two diseases that PAN (polyarteritis nodosa) is associated with
Hep B Hairy cell leukemia *sidenote has also be a/w strep in children, and also a/w minocycline!
896
What is the most commonly positive antibody in anti-phospholipid syndrome? And how abt the most specific?
Anti-cardiolipin is most common Anti-B2-glycoprotein is the most specific
897
Anti-cardiolipin antibodies
Most common positive in APLS *other common Ab are lupus anticoagulant, anti-b2-glycoprotein
898
What drug is known for causing cause livedo reticularis?
Amantidine
899
Most common complication from parotidectomy
Auriculotemporal (Frey syndrome)
900
Mucha-Haberman disease
"Febrile ulceronecrotic Mucha-Haberman disease" is a really severe form of PLEVA that occurs with high fever, lymphadenopathy, arthritis, mucosa stuff, GI *high TNF alpha levels
901
What is the severe form of PLEVA called?
Febrile ulceronecrotic Mucha-Haberman disease
902
Which type of interface dermatitis has no eosinophils
PLEVA and PLC
903
What is the best predictor for speed of disease resolution
Diffuse distribution
904
Most common auto immune disease a/w lichen sclerosis
Autoimmune thyroid
905
Most people with lichen sclerosis have circulating antibodies to what protein?
ECM-1. *ECM-1 also seen in lipoid proteinosis
906
50% of all vulvar SCCs occur in the setting of what disease?
LS&A!
907
What is Schnitzlers syndrome?
Urticarial vasculitis plus monoclonal IgM plus systemic features like fever/bone pain/ect
908
Name of the syndrome: urticarial vasculitis plus IgM antibody plus fever/bone pain
Schnitzlers syndrome (SchMitzlers syndrome)
909
What type of Monoclonal Ab seen in Schnitzlers syndrome?
Monoclonal IgM
910
Which type of lupus carries a risk of SCC?
Hypertrophic Lupus (basically hypertrophic LP on the upper half of the body)
911
What three reasons could you have for getting carotenemia?
1. Eating too many carrots 2. Hypothyroid 3. DM *bc cant convert the vitamin
912
Name a common practice that is a risk factor for DLE
Smoking!
913
What percent of SLE patients will have DLE? | And what percent of DLE patient will progress to SLE?
40% of SLE patients will have DLE lesions But only 5-20% of DLE patients will progress to SLE * fyi 5% risk factor if DLE only on your head * 20% risk factor if DLE is diffuse
914
What percent of DLE patients have mucosal involvement>?
25%
915
ANA is positive in what percent of DLE patients?
Only 5-25%
916
Which type of chronic lupus has an increased risk of SCC?
Hypertrophic LE
917
What is the difference in clinical distribution of hypertrophic LP vs hypertrophic LE? (lupus erythematosus)
Hypertrophic LE favors the UPPER HALF of the body while hypertrophic LP favors the LOWER HALF of the body
918
What is reticular erythematous mucinosis
Histologically similar to Jessners, but is morphological distinctive : erythematous macules and papules on the mid back/chest in a reticular pattern)
919
Treatment for tumid lupus
Tumid lupus is usually a solitary indurated plaque on the face. Treatment includes antimalarials
920
Most specific antibody for Sjögren’s syndrome
Alpha-fodrin
921
Which is more likely to progress to SLE: childhood DLE or adult DLE?
Childhood DLE and widespread DLE have a greater chance of progressing to SLE (20%)
922
How do you tell the difference between Jessners and tumid lupus?
Jessners is a pseudolymphoma and so it has a CD8 infiltrate and no mucin Tumid lupus has a ton of mucin and no CD8
923
What would you choose to treat lupus if topical treatments aren’t working?
First - hydroxychloroquine | Second, can add quinacrine
924
What is the most common systemic finding in SCLE?
Arthralgias (70%)
925
Most common antibody found in SCLE
Anti-Ro/SSA
926
Which kind of SCLE is it rare to have systemic SLE?
Drug induced SCLE has no systemic involvement usually, just skin
927
Name the most common drug that causes drug induced SCLE
HCTZ *other common ones are griseofulvin, terbinafine, calcium channel blockers, eat receipt
928
Which antibody most commonly found in 99% of neonatal lupus?
Anti-Ro/SSA
929
What percent of women undiagnosed SLE women who have Ro Ab will have a child with neonatal lupus? - How abt women wtih known SLE and Ro ab - How abt women w ho have had a prior child with neonatal lupus?
- 1% chance if undiagnosed - 15% chance if have lupus - 25% chance of having a second child with neonatal lupus if you’ve already had one
930
Alternative treatment for LPP
Pioglitazone
931
Drug association with multiple osteoma cutis on a middle aged lady’s face
Tetracyclines (just think about acne and acne treatments)

DERMATOLOGY Premade cards (15 decks)

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