VESICULOBULLOUS DISEASES Flashcards

1
Q

INTRAEPIDERMAL BLISTERING DISEASE

A

PEMPHIGUS VULGARIS (PV)

PEMPHIGUS FOLIACEUS (PF)

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2
Q

SUBEPIDERMAL BLISTERING DISEASES

A

PARANEOPLASTIC PEMPHIGUS

BULLOUS PEMPHIGOID

PEMPHIGUS GESTATIONIS

DERMATITIS HERPETIFORMIS

LINEAR IGA DISEASE

EPIDERMOLYSIS BULLOSA ACQUISITA

CICATRIAL PEMPHIGOID

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3
Q

loss of cohesion between epidermal cells //
loss of keratinocytes

A

acantholysis

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4
Q

Characterized by: loss of cohesion between epidermal cells
(acantholysis)

A

PEMPHIGUS VULGARIS (PV)

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5
Q

PEMPHIGUS VULGARIS (PV) is a blistering disease seen predominantly in __

A

elderly

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6
Q

PICTURES

A

PICTURES

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7
Q

Positive nikolsky sign

A

PEMPHIGUS VULGARIS (PV)

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8
Q

Denuded skin. Beefy red. Involvement of the mucosa (red), flaccid blister in the center with a denuded skin beside it.

A

PEMPHIGUS VULGARIS (PV)

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9
Q

DIF: deposits of IgG on the surface of keratinocytes

A

PEMPHIGUS VULGARIS (PV)

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10
Q

tomb-stone appearance in patient in biopsy using DIF (Direct immunofluorescence)

A

PEMPHIGUS VULGARIS (PV)

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11
Q

pemphigus vulgaris treatment

A

Systemic steroid

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12
Q

Acantholytic blisters are located high within epidermis, just beneath the stratum corneum

A

PEMPHIGUS FOLIACEUS (PF)

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13
Q

More superficial blistering disease

A

PEMPHIGUS FOLIACEUS (PF)

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14
Q

PEMPHIGUS FOLIACEUS (PF) aggravating factor

A

sun exposure

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15
Q

Pemphigus foliaceus drug exposure associated:

A

Drug exposure associated: penicillamine, captopril,
rifampin, piroxicam, penicillin and phenobarbital

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16
Q

Acantholytic mucocutaneous disease associated with an occult or confirmed neoplasm

A

PARANEOPLASTIC PEMPHIGUS

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17
Q

PICTURES

A

PICTURES

18
Q

Lesions: tense blisters on the abdomen, groin, flexor
surfaces of the extremities. Oral mucosa involvement.

A

BULLOUS PEMPHIGOID

19
Q

Biopsy: eosinophil-rich infiltrate within papillary dermis at sites of vesicle formation

A

BULLOUS PEMPHIGOID

20
Q

DIF: Linear deposits of Ig G and/or C3 in the epidermal
basement membrane

A

BULLOUS PEMPHIGOID

21
Q

BULLOUS PEMPHIGOID TREATMENT

A

Systemic glucocorticoids combination azathioprine, cyclophosphamide

22
Q

the following are steroid sparer

A

Azathioprine and Cyclophosphamide

23
Q

PEMPHIGUS GESTATIONIS AKA

A

HERPES GESTATIONIS

24
Q

• Rare, nonviral subepidermal blistering disease of pregnancy and puerperium
• May begin during any trimester of pregnancy or present shortly after delivery

A

PEMPHIGUS GESTATIONIS AKA.
HERPES GESTATIONIS

25
Q

Biopsy: teardrop-shaped subepidermal vesicle with eosinophil-rich leukocytic infiltrate

A

PEMPHIGUS GESTATIONIS AKA.
HERPES GESTATIONIS

26
Q

DIF: Linear deposits of C3 in the epidermal BMZ

A

PEMPHIGUS GESTATIONIS AKA.
HERPES GESTATIONIS

27
Q

Intensely pruritic, papulovesicular skin disease

A

DERMATITIS HERPETIFORMIS

28
Q
  • usually subclinical gluten-sensitive enteropathy
  • may present of any age 2nd to 4th decades most common
    onset
A

DERMATITIS HERPETIFORMIS

29
Q

Biopsy: subepidermal bulla neutrophil-rich infiltrates within dermal papillae

A

DERMATITIS HERPETIFORMIS

30
Q

DIF: confirmatory test – granular IgA deposits in the papillary dermis and along epidermal BMZ

A

DERMATITIS HERPETIFORMIS

31
Q

Confirmatory test for DERMATITIS HERPETIFORMIS

A

DIF: confirmatory test – granular IgA deposits in the papillary dermis and along epidermal BMZ

32
Q

DERMATITIS HERPETIFORMIS MAINSTAY OF TREATMENT

A

• Mainstay of treatment: dapsone (50-200mg/d) – rapid response 24-48 H, hemolysis and methemoglobinemia
• Gluten restriction

33
Q

Needs gluten restriction

A

DERMATITIS HERPETIFORMIS

34
Q

Lesions consists of papulovesicles, bullae and/or urticarial plaques

A

LINEAR IGA DISEASE

35
Q

DIF: linear deposits of IgA and often C3

A

LINEAR IGA DISEASE

36
Q

LINEAR IGA DISEASE TREATMENT

A

Dapsone

37
Q

Often associated with inflammatory bowel disease (Chron’s disease)

A

EPIDERMOLYSIS BULLOSA ACQUISITA

38
Q

Biopsy: subepidermal blister with leukocytic infiltrate

A

EPIDERMOLYSIS BULLOSA ACQUISITA

39
Q

Chronic non-inflammatory form of EPIDERMOLYSIS BULLOSA ACQUISITA (EBA): resistant to treatment but may respond to __

A

IVIG/Cyclosporine

40
Q

erosive lesions of mucous membranes and skin
that result in scarring

A

CICATRIAL PEMPHIGOID

41
Q

Erosive lesions at larynx: hoarseness, pain

A

CICATRIAL PEMPHIGOID