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SDNP exam > Genodermatoses/systemic disease > Flashcards

Genodermatoses/systemic disease Flashcards

1
Q

Hyperkeratotic papules and plaques in intertriginous areas, yellow-brown or reddish, firm coarse like sandpaper on the upper trunk and neck, malodorous in intertriginous areas, may manifest as painful fissures

A

Darier-Darier

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2
Q

Nail manifestations of darier-darier

A

longitudinal broad red/white stripes
v shaped nick at free edge of nail

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3
Q

Palmar pitting and cobblestone pattern of small papules on mucous membranes with gummy overgrowth

A

darier-darier

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4
Q

Symmetrical painful crusted erosions, red and raw blistered, macerated skin fold, resolves w/out scarring, central clearing with lesion expansion

A

Hailey hailey or “benign chronic pemphigus’

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5
Q

what are hailey hailey paitents at risk for?

A

eczema herpeticum
bacterial infection

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6
Q

what triggers darier darier

A

sun exposure, friction/heat, sweat

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7
Q

scaly erythematous red papules pronounced in intertriginous and scalp

A

langerhans histiocytosis

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8
Q

treatment of Langerhans histiocystosis

A

hydroxyurea
methotrexate

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9
Q

Affects one leg, buttock to foot, spreads over months-years birth to 5 years, present in the lines of blaschko

A

Birthmark due to overgrowth of the epidermis (ILVEN)

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10
Q

If you diagnose keratoderma, what should you do

A

genetic workup w/ biopsy

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11
Q

treatment goals of palmoplantar keratoderma

A

Soften the thicken skin.

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12
Q

Tuberous Sclerosis Complex

A

inherited autosomal dominant diseases

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13
Q

presents 3-10yr old
small pink or red spots in butterfly distribution

A

NF1

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14
Q

Lisch nodules
6 or more cafe au lait
multiple Neurofibroma

A

NF1

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15
Q

Shagreen patch is the hallmark-flesh colored or orange peel connective tissue nevi, most common in lower back

A

Tuberous sclerosis

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16
Q

Nail findings of TSC

A

smooth firth flesh colored emerging from nail fold, longitudinal groove in the nail, splinter hemorrhage

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17
Q

Ash leaf marks are the hall mark sign of ? (white ash leaf shaped marks)

A

TSC

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18
Q

Ichthyosis hallmark sign

A

Fish scale surprise, dry thickened scaling skin

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19
Q

present at birth
mutation in filaggrin gene
increased association with atopic triad/keratosis pilaris
hyperlinearity of palms/soles

A

Ichythosis vulgaris

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20
Q

X linked icthyosis puts patient at high risk of

A

higher risk of cryptorchism and testicular

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21
Q

Painful hyperkeratosis involving the hair, nails or mucosal surfaces, palmoplantar hyperhidrosis

A

PPK

soften skin with keratolytics, exfoliative- urea 20-40%

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22
Q

ash-leaf spots- hypopigmented macules
angiofibromas- bilateral face
shagreen patches- connected tissue nevi usually on lower trunk
forehead fibrous plaques (yellow tan plaques)
ungual fibromas under nail plate
extracutaneous brain lesion, dental abnorm, cardiac, ocular, pulmonary, hepatic and renal manifestations

A

TSC

goal is early ID and referral

23
Q

How to treat Darier-Darier?

A

off label treatments include

retinoids, corticosteroid

24
Q

How to treat Hailey Hailey

A

clindamycin or mupirocin x 4 weeks
topical keto cream for suspected secondary fungal infections
TCS &/or TCIs

25
Q

yellowish flat papules/plaques most often upper eyelids (trunk,neck, axillae)

if occurs btw digits, sign of familial

A

xanthomas/xanthelasma

fasting lipids

26
Q

reactivation of latent varicella zoster virus
>4 months after resolution of skin eruption
most common in a facial eruption
sharp, stabbing burning pain; constant or intermittent

A

postherpetic neuralgia

gabapentin or pregabalin
mild topical capsaicin or lidocaine for short term relief

Moderate to severe: carbamazepine, amitriptylline, desipramine, fluoxetine, or paroxetine

27
Q

Usually from vertebral column disease, circumscribed area of back, usually scapular border, midback, hyperpigmentation

A

NOTALGIA PARASTHETICA

capsaicin cream/patch
gabapentin
TCS
botox

28
Q

Patient presents with a hx of back and neck pain with complaints of itching, burning and stinging on the dorsolateral forearm, symptoms worsen with heat, improve with cold?

A

brachioradial pruritus

TX with:
topical capsaicin, gabapentin, fluoxetine, amitriptyline, doxepin

29
Q

Patient complains of anogenital itch, no rash is present?

A

neuropathic anogenital pruritus

30
Q

Impacts patient QOL, symmetrical hyperkeratotic, excoriated nodules and papules
tingling and stinging present

A

Prurigo Nodularis

dupixent- 2022

TCS

SSRIs, TCAs, antiseizure drugs, sedatives, psychotherapy

31
Q

pruritus without primary lesions, often secondary lesions from scratching.

Workup:

A

Medical HX
CBCw/diff
lft
renal panel
Thyroid function test
skin biopsy H&E
skin biopsy for DIF and serum for ELISA

32
Q

Great imitator

A

sarcoidosis

33
Q

often erupted in scars, tattoos, facial keloids.
lupus pernio in nose and cheeks
lacrimal gland enlargement
APPLE JELLY BROWN YELLOW APPEARANCE
thick smooth plaques with little if any epidermal changes

A

Sarcoidosis

biopsy will reveal non-caseating granulomas superficial and deep dermis; macrophages and histiocytes.

34
Q

If there is sarcoidosis involvement near nose/mouth there is

A

high risk 90-95% of pulmonary involvement

35
Q

strong association with DM
Multiple pink/brown/yellow plaques w/ atrophy, telangiectasia and ulcerations common on pretibial area,
commonly young adult and middle age women.

A

Necrobiosis Lipoidica

full thickness punch biopsy

treat with cortico steroids (ILK & TOPICAL)

36
Q

Woman in her 30s-60s will present with very sick appearance, febrile with sudden onset of painful, juicy plaques, vesicles & bullae on the face, neck and dorsal hands.

What is your dx?
How will you treat?

A

Sweet’s syndrome

systemic corticosteroids for quick relief

dapsone for severe/recalcitrant

37
Q

What medications can cause sweets syndrome?
what disease processes and surgeries can cause?

A
  • isotretinoin, ocp, furosemide, tetracycline, hydralazine, bactrim

-Intestinal bypass surgery, URI, Strep infection, Hashimoto’s thyroiditis, Sjogren’s syndrome

38
Q

Patient presents with a shallow ulceration that has spread quickly, reports pain.

Upon examination you find a purulent base with necrosis, a border that expands centrifugally with underminded edges?

What do you suspect and what should you do next?

A

PG

DO NOT SURGICALLY DEBRIDE.

  • first line topical, ILK or systemic corticosteroid

Referral to specialist including wound care, gastroenterology, hematology

39
Q

most common skin CA
slow growing, uncommon to metastasize

inactivation of pTCH tumor suppression gene
mutations in hedgehog pathway

A

Basal Cell CA

40
Q

pink, smooth pearly papule/nodule with rolled border, telangiectasia or arborizing blood vessels, ulceration/pigmentation may be present

A

Nodular BCC

41
Q

butterfly rash with erythematous pathches/plaques with bullous, discoid or erosions

A

acute cutaneous LE

42
Q

annular, polycyclic w/ central clearing and psoriasiform, not below the waist

A

subacute cutaneous LE

43
Q

DX testing for lupus erythematous

A

punch w/ dif
CBC w/ diff, sed rate, ana panel, UA

44
Q

1st line treatment for LE

A

hydroxychloroquine, bt has decreased efficacy in smokers

45
Q

Do not give these patients hydroxychloroquine

A

g6pd insufficiency
myasthenia gravis

46
Q

what drugs commonly cause drug induced lupus erythematous

A

hydralazine, procainimide, anticonvulsants, minocycline, sulfonamides

47
Q

polycyclic, annular or bullous rash on scalp or periorbital (owl eyes)

A

Neonatal lupus

48
Q

coin shapes thick, dark, scaly plaques
present on face, scalp, conchal bowl, palate,
CARPET TACK SCALE

A

DLE

biopsy for H&E

no FDA approved treatment

49
Q

why is lupus pernio pertinent finding?

A

cutaneous sarcoidosis finding

50
Q

circumscribed ivory colored, sclerotic fixed plaques with violaceous border, face (en coup de sabre), linear extremities/forehead

TX with:
topical corticosteroids
Off label MTX, hydroxychloroquine, mycophenolate

A

LOCALIZED SCLERODERMA

51
Q

What does CREST stand for

A

calcinosis, raynaud’s esophageal dysfunction, sclerodactyly, telangiectasia

52
Q

heliotrope and gottrons papules

A

dermatomyositis

53
Q

When is there poor prognosis with dermatomyositis

A

muscle weakness present for > 4 months

54
Q

insidious onset of proximal muscle weakness, diffuse hair loss, periungal erythema w/ telangiectasia, red scaly scalp/frontal hairline, extremely pruritic, violaceous scaly plaques on the trunk (shawl sign), photosensitivity,

A

dermatomyositis

H&E and dif

SDNP exam (14 decks)

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