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Flashcards in Vascular diseases of the skin Deck (156):
1

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

BPAG1 and BPAG2

2

What is the pathophysiology of bullous pemphigoid?

Attack against the BPAG1 and BPAG2

3

What are the three major locations for blisters?

-Subcorneal
-Intraepidermal
-Subepidermal

4

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

Abs against patient's perilesional skin helps diagnose

5

What is indirect immunofluorescence tool to diagnose skin disorders?

Circulating Ab in patient's serum against normal skin

6

What is the most common bullous autoimmune disease?

Bullous pemphigoid

7

What is Bullous pemphigoid?

Type II hypersensitivity, Blistering skin disease

8

What age range is generally affected with bullous pemphigoid?

60-80+

9

Which gender is usually more affected with bullous pemphigoid?

Equal

10

What is the pathophysiology of bullous pemphigoid?

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

11

What are the skin findings with bullous pemphigoid?

Urticarial/papular lesions that progress to tense bullae

12

What are the s/sx of bullous pemphigoid?

-Urticarial/papular lesions
-Itchy
-bullae arise in normal or erythematous skin

13

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

Mucosal surfaces

14

What is the natural history of bullous pemphigoid?

Evolves over weeks/months to a generalized bullous eruption

15

Tense bullae arising in tense or erythematous skin = ?

bullous pemphigoid

16

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

bullous pemphigoid does NOT involved mucosal surfaces

Pemphigus does

17

What type of hypersensitivity reaction is bullous pemphigoid?

Type II

18

What is erythrodermic bullous pemphigoid?

Body wide distribution of bullous pemphigoid

19

What is spongiosus?

A lot of edema between cells of the epidermis

20

What is the initial histological finding of bullous pemphigoid?

Inflammation of the epidermis, with eosinophils surrounding

21

What is the direct immunofluorescence finding in bullous pemphigoid?

Linear deposition of IgG and C3 along the BM zone

22

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

bullous pemphigoid

23

What is the treatment for bullous pemphigoid?

Systemic steroids or other immunosuppressants

If local, then local steroids

24

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

False--no scarring

25

How do you follow a patient with bullous pemphigoid?

Serum antibodies to BP180 and BP230

26

What is cicatricial pemphigoid?

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

27

What is the natural h/o cicatricial pemphigoid?

Chronic and recurring course

28

True or false: there is often scarring with cicatricial pemphigoid

True

29

What is the treatment for cicatricial pemphigoid?

Immunosuppressives

30

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

Look at mucosal surfaces

31

What are the differences between cicatricial pemphigoid and bullous pemphigoid?

none

32

What is pemphigoid gestationis?

Rare, intensely pruritic dermatosis of pregnancy

33

What are the skin findings with pemphigoid gestationis?

vesicular eruptions on abdomen with sparing of mucous membranes

34

When in gestation does pemphigoid gestationis often occur?

4th-7th month

35

Which ethnicity is usually affected with pemphigoid gestationis?

Whites

36

True or false: pemphigoid gestationis can recur in subsequent pregnancies

True

37

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

5%
Typically resolves 6 months postpartum

38

What is the management for pemphigoid gestationis?

Systemic prednisone to suppress blisters and relieve itching

39

Bullous lesions affecting the abdomen of a pregnant woman = ?

pemphigoid gestationis

40

Does pemphigoid gestationis involved the umbilicus?

Yes--if not, think of other things

41

What are the histological findings of pemphigoid gestationis?

Same as bullous pemphigoid

42

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

Appears in the striae, and is umbilical SPARING
-no blisters

43

What is the histology like with PUPP?

Dermatitis-like

44

What are the DIF findings in PUPP?

Negative

45

What is pregnancy prurigo?

Non-blistering skin disorder that arises in pregnancy

46

What are the histological findings of pregnancy prurigo?

Dermatitis pathology

47

What are the DIF findings in pregnancy prurigo?

Negative

48

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

20-60 year old males

49

What are the skin findings of dermatitis herpetiformis?

-Papules
-Vesicles
-Occasional bullae on an erythematous, often urticarial base

50

Is dermatitis herpetiformis pruritic?

Yes

51

What is the treatment for dermatitis herpetiformis? (2)

-Gluten free diet
-Dapsone

52

What disease is dermatitis herpetiformis commonly associated with?

Celiac disease

53

Which ethnicity is affected with dermatitis herpetiformis?

Caucasians

54

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

Extensor surfaces of elbow and knees

55

What is the pathogenesis of dermatitis herpetiformis?

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

56

How can you follow the course of dermatitis herpetiformis?

Follow TTG antibodies

57

What are the histological findings of dermatitis herpetiformis?

Subtle subepidermal blisters, filled with PMNs

58

What are the DIF findings of dermatitis herpetiformis?

Granular IgA deposition in dermal papillae

59

What is adult linear bullous dermatosis?

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

60

What is childhood linear bullous dermatosis?

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

61

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

Trunk and mucous membranes

62

What are the skin findings of linear bullous dermatosis?

Group vesicles ("cluster of jewels")

63

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

linear bullous dermatosis

64

What is the drug that can cause linear bullous dermatosis?

Vancomycin

65

What are the histological findings of linear bullous dermatosis?

Subepidermal papillae filled with PMNs

66

What are the DIF findings of linear bullous dermatosis?

Linear deposition of IgA along the BM

67

Linear deposition of IgA along the BM on DIF = ?

linear bullous dermatosis

68

What is the most common type of porphyria?

Porphyria cutanea tarda (PCT)

69

What is the enzyme that is defective in PCT?

Uroporphyrinogen decarboxylase

70

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

Porphobilinogen deaminase
AD

71

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

Uroporphyrins and coproporphyrins

Absorb light to cause a phototoxic effects

72

What hematological disease can predispose patients to PCT?

Hemochromatosis genes

73

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

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

What are the environmental exposures that contribute to PCT?

EtOH and estrogens

75

What infectious agent is associated with PCT?

Hep C

76

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

Flaviviridae
+ssRNA
Enveloped

77

What areas of the skin are usually affected with PCT?

Blisters on light exposed skin, particularly the hands

78

What are the skin findings of PCT?

Tense bullae on normal appearing background

79

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

PCT

80

What are the urine findings of PCT?

Fluoresce red

81

What is the histology of PCT

Cell poor subepidermal blisters

82

What are the DIF findings ot PCT?

Superficial blood vessels with circumferential IgG deposition

83

What is the management for PCT?

Total avoidance of EtOH and estrogens
-Phlebotomy to reduce Fe
-Low dose chloroquine

84

What is the MOA of chloroquine?

Causes a toxic buildup of heme in RBCs to kill parasites

85

What is pseudoporphyria?

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

86

In whom does pseudoporphyria usually occur?

Dialysis patients

87

What is the histology of pseudoporphyria?

same as PCT

88

What are the drugs that typically lead to pseudoporphyria?

NSAIDs
Tetracyclines

89

What is bullous diabeticorum?

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

90

True or false: there is often scarring with bullous diabeticorum

False

91

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

Legs and feet

92

What are the DIF findings of bullous diabeticorum?

Negative

93

What are the histological findings of bullous diabeticorum?

Often cell poor subepidermal blisters, but variable blisters

94

What is pemphigus vulgaris?

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

95

What is the age range typically affected with pemphigus vulgaris?

40-60

96

Which gender is more commonly affected with pemphigus vulgaris?

Equal

97

What ethnicities are most commonly affected with pemphigus vulgaris?

Jewish/mediterranean

98

What is the natural h/o pemphigus vulgaris?

Chronic course with acute exacerbations and remissions

Fatal if untreated

99

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

50%

100

What are the skin findings of pemphigus vulgaris?

Progression from erythema to blisters to ulceration
-Blisters are flaccid and arise on normal skin

101

How do you treat pemphigus vulgaris?

Immunosuppressives

102

What is the pathophysiology of pemphigus vulgaris?

Autoantibodies against desmoglein 1 and 3

103

What are desmosomes?

Between cells

104

Where are hemidesmosomes?

Between cell and BM

105

How can you follow the course of pemphigus vulgaris?

DSG1 and DSG3 antibody titers

106

Where are the blisters of pemphigus vulgaris located?

Often start in the oral mucosa

107

Are the blisters with pemphigus vulgaris pruritic or painful?

Not pruritic, but painful

108

What is Nikolsky sign?

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

109

Flaccid vesicles on normal skin = ?

pemphigus vulgaris

110

Tense vesicles on erythematous skin = ?

Bullous Pemphigoid

111

What are the histological findings of pemphigus vulgaris?

Interepidermal blister

112

What are the DIF findings of pemphigus vulgaris?

Cell surface IgG or C3 "chicken wire"

113

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

-Staph aureus or strep pyogenes
-toxin that cleaves desmoglein 1

114

What are the skin findings of impetigo?

honey crusted lesions

115

What are the histological findings of impetigo?

Superficial blister with focal acantholysis

116

What are the histological characteristics of friction blisters?

Cell poor blister below the stratum corneum

117

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

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

118

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

10-40

119

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

Vesicles and bullae replaced by crusting erosions resembling impetigo

120

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

Flexural and intertriginous areas (axillae, groin, neck)

121

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

Tetracycline and topical steroids

122

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

ATP2C1

123

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

Acantholysis forming a dilapidated brick wall

124

What is acantholysis?

Loss of intercellular adhesions (e.g. desmosomes)

125

Acantholysis forming a dilapidated brick wall appearance on histology = ?

Hailey-Hailey disease (benign familial pemphigus)

126

What is Darier's disease?

AD defect in ATP2A2--a Ca pump

127

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

0-20

128

What are the skin findings of Darier's disease?

Multiple discrete scaling papules in seborrheic distribution

129

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

-Sunscreens
-Avoid rubbing
-Retinoids

130

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

Chest
Back
ears
Forehead
Scalp
Groin

131

Multiple discrete scaling papules in seborrheic distribution = ?

Darier's disease

132

What are the histological findings of darier's disease?

Total epidermal acantholysis

133

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

Middle aged and older
M more than F

134

What causes Grover's disease?

heavy sweating / heat, usually from bedridden patients

135

What are the skin findings of Grover's disease?

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

136

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

trunk and proximal extremities

137

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

Grover's disease

138

What is the treatment for Grover's disease?

Topical steroids or phototherapy

139

What are the histological findings of Grover's disease?

Acantholysis

140

Cell poor or cell rich (inflammatory): PCT

Cell poor

141

Cell poor or cell rich (inflammatory): TEN

Cell poor

142

Cell poor or cell rich (inflammatory): Bullous pemphigoid

Inflammatory

143

Cell poor or cell rich (inflammatory): dermatitis herpetifromis

Inflammatory

144

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

Inflammatory

145

Cell poor or cell rich (inflammatory): bullous SLE

Inflammatory

146

Subepidermal, Subcorneal, or Suprabasal: pemphigus foliaceus

Sub corneal

147

Subepidermal, Subcorneal, or Suprabasal: SSSS

Subcorneal

148

Subepidermal, Subcorneal, or Suprabasal: impetigo

Subcorneal

149

Subepidermal, Subcorneal, or Suprabasal: herpes

Suprabasal

150

Subepidermal, Subcorneal, or Suprabasal: pemphigus vulgaris

Suprabasal

151

Subepidermal, Subcorneal, or Suprabasal: pemphigus vegetans

Suprabasal

152

Subepidermal, Subcorneal, or Suprabasal: acantholytic disorders

Suprabasal

153

Subepidermal, Subcorneal, or Suprabasal: PCT

Subepidermal

154

Subepidermal, Subcorneal, or Suprabasal: linear IgA dermatosis

Subepidermal

155

Subepidermal, Subcorneal, or Suprabasal: TEN/SJS

Subepidermal

156

Subepidermal, Subcorneal, or Suprabasal: bullous pemphigoid

Subepidermal