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Specialty Medicine > Vascular diseases of the skin > Flashcards

Vascular diseases of the skin Flashcards

1
Q

What are the two antigens that hold the epidermis to the BM?

A

BPAG1 and BPAG2

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2
Q

What is the pathophysiology of bullous pemphigoid?

A

Attack against the BPAG1 and BPAG2

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3
Q

What are the three major locations for blisters?

A
  • Subcorneal
  • Intraepidermal
  • Subepidermal
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4
Q

What is the direct immunofluorescence tool used to diagnose skin disorders?

A

Abs against patient’s perilesional skin helps diagnose

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5
Q

What is indirect immunofluorescence tool to diagnose skin disorders?

A

Circulating Ab in patient’s serum against normal skin

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6
Q

What is the most common bullous autoimmune disease?

A

Bullous pemphigoid

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7
Q

What is Bullous pemphigoid?

A

Type II hypersensitivity, Blistering skin disease

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8
Q

What age range is generally affected with bullous pemphigoid?

A

60-80+

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9
Q

Which gender is usually more affected with bullous pemphigoid?

A

Equal

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10
Q

What is the pathophysiology of bullous pemphigoid?

A

Autoantibody against antigens BP180 (BPAG1) and BP230 (BPAG2)

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11
Q

What are the skin findings with bullous pemphigoid?

A

Urticarial/papular lesions that progress to tense bullae

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12
Q

What are the s/sx of bullous pemphigoid?

A
  • Urticarial/papular lesions
  • Itchy
  • bullae arise in normal or erythematous skin
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13
Q

What areas of the skin are not affected with bullous pemphigoid?

A

Mucosal surfaces

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14
Q

What is the natural history of bullous pemphigoid?

A

Evolves over weeks/months to a generalized bullous eruption

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15
Q

Tense bullae arising in tense or erythematous skin = ?

A

bullous pemphigoid

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16
Q

What is the major difference in skin involvement between bullous pemphigoid and bullous pemphigus?

A

bullous pemphigoid does NOT involved mucosal surfaces

Pemphigus does

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17
Q

What type of hypersensitivity reaction is bullous pemphigoid?

A

Type II

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18
Q

What is erythrodermic bullous pemphigoid?

A

Body wide distribution of bullous pemphigoid

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19
Q

What is spongiosus?

A

A lot of edema between cells of the epidermis

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20
Q

What is the initial histological finding of bullous pemphigoid?

A

Inflammation of the epidermis, with eosinophils surrounding

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21
Q

What is the direct immunofluorescence finding in bullous pemphigoid?

A

Linear deposition of IgG and C3 along the BM zone

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22
Q

Linear deposition of IgG and C3 along the BM zone = ?

A

bullous pemphigoid

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23
Q

What is the treatment for bullous pemphigoid?

A

Systemic steroids or other immunosuppressants

If local, then local steroids

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24
Q

True or false: there is often a lot of skin scarring with bullous pemphigoid

A

False–no scarring

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25
Q

How do you follow a patient with bullous pemphigoid?

A

Serum antibodies to BP180 and BP230

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26
Q

What is cicatricial pemphigoid?

A

Rare variant of bullous pemphigoid of the elderly that affects mucosal surfaces, usually of the head and neck.

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27
Q

What is the natural h/o cicatricial pemphigoid?

A

Chronic and recurring course

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28
Q

True or false: there is often scarring with cicatricial pemphigoid

A

True

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29
Q

What is the treatment for cicatricial pemphigoid?

A

Immunosuppressives

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30
Q

What should always be done when a patient complains of pruritus anywhere?

A

Look at mucosal surfaces

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31
Q

What are the differences between cicatricial pemphigoid and bullous pemphigoid?

A

none

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32
Q

What is pemphigoid gestationis?

A

Rare, intensely pruritic dermatosis of pregnancy

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33
Q

What are the skin findings with pemphigoid gestationis?

A

vesicular eruptions on abdomen with sparing of mucous membranes

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34
Q

When in gestation does pemphigoid gestationis often occur?

A

4th-7th month

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35
Q

Which ethnicity is usually affected with pemphigoid gestationis?

A

Whites

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36
Q

True or false: pemphigoid gestationis can recur in subsequent pregnancies

A

True

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37
Q

What percent of babies born to mother with pemphigoid gestationis develop the skin disease? What is the prognosis?

A

5%

Typically resolves 6 months postpartum

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38
Q

What is the management for pemphigoid gestationis?

A

Systemic prednisone to suppress blisters and relieve itching

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39
Q

Bullous lesions affecting the abdomen of a pregnant woman = ?

A

pemphigoid gestationis

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40
Q

Does pemphigoid gestationis involved the umbilicus?

A

Yes–if not, think of other things

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41
Q

What are the histological findings of pemphigoid gestationis?

A

Same as bullous pemphigoid

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42
Q

What is pruritic papules and plaques of pregnancy? How can you differentiate this from pemphigoid gestationis?

A

Appears in the striae, and is umbilical SPARING

-no blisters

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43
Q

What is the histology like with PUPP?

A

Dermatitis-like

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44
Q

What are the DIF findings in PUPP?

A

Negative

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45
Q

What is pregnancy prurigo?

A

Non-blistering skin disorder that arises in pregnancy

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46
Q

What are the histological findings of pregnancy prurigo?

A

Dermatitis pathology

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47
Q

What are the DIF findings in pregnancy prurigo?

A

Negative

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48
Q

What is the age range and gender that is usually affected with dermatitis herpetiformis?

A

20-60 year old males

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49
Q

What are the skin findings of dermatitis herpetiformis?

A
  • Papules
  • Vesicles
  • Occasional bullae on an erythematous, often urticarial base
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50
Q

Is dermatitis herpetiformis pruritic?

A

Yes

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51
Q

What is the treatment for dermatitis herpetiformis? (2)

A
  • Gluten free diet

- Dapsone

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52
Q

What disease is dermatitis herpetiformis commonly associated with?

A

Celiac disease

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53
Q

Which ethnicity is affected with dermatitis herpetiformis?

A

Caucasians

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54
Q

What area of the body is usually affected with dermatitis herpetiformis, that other skin diseases do not usually affect?

A

Extensor surfaces of elbow and knees

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55
Q

What is the pathogenesis of dermatitis herpetiformis?

A

Antibodies against tissue transglutaminase (TTG) bing to TTG in the gut, circulate to the skin, and activate PMNs

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56
Q

How can you follow the course of dermatitis herpetiformis?

A

Follow TTG antibodies

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57
Q

What are the histological findings of dermatitis herpetiformis?

A

Subtle subepidermal blisters, filled with PMNs

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58
Q

What are the DIF findings of dermatitis herpetiformis?

A

Granular IgA deposition in dermal papillae

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59
Q

What is adult linear bullous dermatosis?

A

An acquired, autoimmune blistering disease that may present with a clinical pattern of vesicles indistinguishable from dermatitis herpetiformis, or with vesicles and bullae in a bullous pemphigoid-like appearance

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60
Q

What is childhood linear bullous dermatosis?

A

An acquired, self-limited bullous disease that may begin by the time the patient is age 2 to 3 and usually remits by age 13

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61
Q

What areas of the body are affected with linear bullous dermatosis?

A

Trunk and mucous membranes

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62
Q

What are the skin findings of linear bullous dermatosis?

A

Group vesicles (“cluster of jewels”)

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63
Q

Group vesicles (“cluster of jewels”) on the skin = ?

A

linear bullous dermatosis

64
Q

What is the drug that can cause linear bullous dermatosis?

A

Vancomycin

65
Q

What are the histological findings of linear bullous dermatosis?

A

Subepidermal papillae filled with PMNs

66
Q

What are the DIF findings of linear bullous dermatosis?

A

Linear deposition of IgA along the BM

67
Q

Linear deposition of IgA along the BM on DIF = ?

A

linear bullous dermatosis

68
Q

What is the most common type of porphyria?

A

Porphyria cutanea tarda (PCT)

69
Q

What is the enzyme that is defective in PCT?

A

Uroporphyrinogen decarboxylase

70
Q

What is the enzyme that is defective in AIP? inheritance pattern?

A

Porphobilinogen deaminase

AD

71
Q

What are the compounds that build up in PCT? What is the effect of this in the skin?

A

Uroporphyrins and coproporphyrins

Absorb light to cause a phototoxic effects

72
Q

What hematological disease can predispose patients to PCT?

A

Hemochromatosis genes

73
Q

What is the protein that is defective in hemochromatosis? How is it defective?

A

HFE

HFE works to increase the intestinal release of iron into the blood. When HFE is mutated, the intestines perpetually interpret a strong transferrin signal as if the body were deficient in iron. This leads to maximal iron absorption from ingested foods and iron overload in the tissues.

74
Q

What are the environmental exposures that contribute to PCT?

A

EtOH and estrogens

75
Q

What infectious agent is associated with PCT?

A

Hep C

76
Q

What is the viral family of Hep C? Genetic makeup? Enveloped?

A

Flaviviridae
+ssRNA
Enveloped

77
Q

What areas of the skin are usually affected with PCT?

A

Blisters on light exposed skin, particularly the hands

78
Q

What are the skin findings of PCT?

A

Tense bullae on normal appearing background

79
Q

Bullae on light exposed areas, (particularly the hands) on normal background of skin = ?

A

PCT

80
Q

What are the urine findings of PCT?

A

Fluoresce red

81
Q

What is the histology of PCT

A

Cell poor subepidermal blisters

82
Q

What are the DIF findings ot PCT?

A

Superficial blood vessels with circumferential IgG deposition

83
Q

What is the management for PCT?

A

Total avoidance of EtOH and estrogens

  • Phlebotomy to reduce Fe
  • Low dose chloroquine
84
Q

What is the MOA of chloroquine?

A

Causes a toxic buildup of heme in RBCs to kill parasites

85
Q

What is pseudoporphyria?

A

Photodistributed blisters resembling PCT, but in the absence of serum, urine, or other porphyrin abnormalities

86
Q

In whom does pseudoporphyria usually occur?

A

Dialysis patients

87
Q

What is the histology of pseudoporphyria?

A

same as PCT

88
Q

What are the drugs that typically lead to pseudoporphyria?

A

NSAIDs

Tetracyclines

89
Q

What is bullous diabeticorum?

A

Weakness of the BM between the dermis and epidermis leads to bullae

90
Q

True or false: there is often scarring with bullous diabeticorum

A

False

91
Q

What part of the body is most often affected with bullous diabeticorum?

A

Legs and feet

92
Q

What are the DIF findings of bullous diabeticorum?

A

Negative

93
Q

What are the histological findings of bullous diabeticorum?

A

Often cell poor subepidermal blisters, but variable blisters

94
Q

What is pemphigus vulgaris?

A

Formation of autoantibodies to the intercellular layers of the epidermis, leading to spontaneous blistering or fissures resulting from minor trauma

95
Q

What is the age range typically affected with pemphigus vulgaris?

A

40-60

96
Q

Which gender is more commonly affected with pemphigus vulgaris?

A

Equal

97
Q

What ethnicities are most commonly affected with pemphigus vulgaris?

A

Jewish/mediterranean

98
Q

What is the natural h/o pemphigus vulgaris?

A

Chronic course with acute exacerbations and remissions

Fatal if untreated

99
Q

What percent of cases of pemphigus vulgaris have oral mucosal involvement?

A

50%

100
Q

What are the skin findings of pemphigus vulgaris?

A

Progression from erythema to blisters to ulceration

-Blisters are flaccid and arise on normal skin

101
Q

How do you treat pemphigus vulgaris?

A

Immunosuppressives

102
Q

What is the pathophysiology of pemphigus vulgaris?

A

Autoantibodies against desmoglein 1 and 3

103
Q

What are desmosomes?

A

Between cells

104
Q

Where are hemidesmosomes?

A

Between cell and BM

105
Q

How can you follow the course of pemphigus vulgaris?

A

DSG1 and DSG3 antibody titers

106
Q

Where are the blisters of pemphigus vulgaris located?

A

Often start in the oral mucosa

107
Q

Are the blisters with pemphigus vulgaris pruritic or painful?

A

Not pruritic, but painful

108
Q

What is Nikolsky sign?

A

Dislodging of epidermis by lateral fingers pressure near lesions, leading to erosions

109
Q

Flaccid vesicles on normal skin = ?

A

pemphigus vulgaris

110
Q

Tense vesicles on erythematous skin = ?

A

Bullous Pemphigoid

111
Q

What are the histological findings of pemphigus vulgaris?

A

Interepidermal blister

112
Q

What are the DIF findings of pemphigus vulgaris?

A

Cell surface IgG or C3 “chicken wire”

113
Q

What infectious agent causes impetigo? How do they cause it?

A
  • Staph aureus or strep pyogenes

- toxin that cleaves desmoglein 1

114
Q

What are the skin findings of impetigo?

A

honey crusted lesions

115
Q

What are the histological findings of impetigo?

A

Superficial blister with focal acantholysis

116
Q

What are the histological characteristics of friction blisters?

A

Cell poor blister below the stratum corneum

117
Q

What is Hailey-Hailey disease (benign familial pemphigus)?

A

AD defect in ATP2C1, a Ca pump leads to impairment of inter-cellular stores of Ca, and decreased cellular cohesion

118
Q

What is the age range that is typically affected with Hailey-Hailey disease (benign familial pemphigus)?

A

10-40

119
Q

What are the skin findings of Hailey-Hailey disease (benign familial pemphigus)?

A

Vesicles and bullae replaced by crusting erosions resembling impetigo

120
Q

Where are the vesicles/bullae found on the skin with Hailey-Hailey disease (benign familial pemphigus)?

A

Flexural and intertriginous areas (axillae, groin, neck)

121
Q

What is the treatment for Hailey-Hailey disease (benign familial pemphigus)?

A

Tetracycline and topical steroids

122
Q

What is the protein that is defective in Hailey-Hailey disease (benign familial pemphigus)?

A

ATP2C1

123
Q

What are the histological findings of Hailey-Hailey disease (benign familial pemphigus)?

A

Acantholysis forming a dilapidated brick wall

124
Q

What is acantholysis?

A

Loss of intercellular adhesions (e.g. desmosomes)

125
Q

Acantholysis forming a dilapidated brick wall appearance on histology = ?

A

Hailey-Hailey disease (benign familial pemphigus)

126
Q

What is Darier’s disease?

A

AD defect in ATP2A2–a Ca pump

127
Q

What is the age range that is affected with Darier’s disease?

A

0-20

128
Q

What are the skin findings of Darier’s disease?

A

Multiple discrete scaling papules in seborrheic distribution

129
Q

What is the treatment for Darier’s disease? (3)

A
  • Sunscreens
  • Avoid rubbing
  • Retinoids
130
Q

Where on the skin are blisters with Darier’s disease usually found?

A 
Chest
Back
ears
Forehead
Scalp
Groin
131
Q

Multiple discrete scaling papules in seborrheic distribution = ?

A

Darier’s disease

132
Q

What are the histological findings of darier’s disease?

A

Total epidermal acantholysis

133
Q

What is the age range that is usually affected with Grover’s disease? Which gender?

A

Middle aged and older

M more than F

134
Q

What causes Grover’s disease?

A

heavy sweating / heat, usually from bedridden patients

135
Q

What are the skin findings of Grover’s disease?

A

Abrupt onset of pruritic crops of reddish, small papules with vesicles and erosions

136
Q

What areas of the body are affected with Grover’s disease?

A

trunk and proximal extremities

137
Q

Abrupt onset of pruritic crops of reddish, small papules with vesicles and erosions
= ?

A

Grover’s disease

138
Q

What is the treatment for Grover’s disease?

A

Topical steroids or phototherapy

139
Q

What are the histological findings of Grover’s disease?

A

Acantholysis

140
Q

Cell poor or cell rich (inflammatory): PCT

A

Cell poor

141
Q

Cell poor or cell rich (inflammatory): TEN

A

Cell poor

142
Q

Cell poor or cell rich (inflammatory): Bullous pemphigoid

A

Inflammatory

143
Q

Cell poor or cell rich (inflammatory): dermatitis herpetifromis

A

Inflammatory

144
Q

Cell poor or cell rich (inflammatory): linear IgA dermatosis

A

Inflammatory

145
Q

Cell poor or cell rich (inflammatory): bullous SLE

A

Inflammatory

146
Q

Subepidermal, Subcorneal, or Suprabasal: pemphigus foliaceus

A

Sub corneal

147
Q

Subepidermal, Subcorneal, or Suprabasal: SSSS

A

Subcorneal

148
Q

Subepidermal, Subcorneal, or Suprabasal: impetigo

A

Subcorneal

149
Q

Subepidermal, Subcorneal, or Suprabasal: herpes

A

Suprabasal

150
Q

Subepidermal, Subcorneal, or Suprabasal: pemphigus vulgaris

A

Suprabasal

151
Q

Subepidermal, Subcorneal, or Suprabasal: pemphigus vegetans

A

Suprabasal

152
Q

Subepidermal, Subcorneal, or Suprabasal: acantholytic disorders

A

Suprabasal

153
Q

Subepidermal, Subcorneal, or Suprabasal: PCT

A

Subepidermal

154
Q

Subepidermal, Subcorneal, or Suprabasal: linear IgA dermatosis

A

Subepidermal

155
Q

Subepidermal, Subcorneal, or Suprabasal: TEN/SJS

A

Subepidermal

156
Q

Subepidermal, Subcorneal, or Suprabasal: bullous pemphigoid

A

Subepidermal

Specialty Medicine (36 decks)

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