CHAPTER 21: CHRONIC BLISTERING DERMATOSES

Question 1

characterized by mucosal erosions and by thin-walled, relatively flaccid, easily ruptured bullae that appear on apparently normal skin and mucous mem- branes or on erythematous bases

First appear in the mouth

+ nikolsky and asboe-hansen sign

ruptured bullae with painful erosions

antibodies against desmoglein 1 and 3 ( muscosal and skin involvement)

antibodies against desmoglein 3 ( mucosal dominant)

Answer 1

Pemphigus vulgaris (PV)

Question 2

tx of phemphigus vulgaris

Answer 2

1. Corticosteroids: Prednisone
2. Rituximab
3. Oral Immunosuppressive Agents: Azathioprine, Mycophenolate Mofetil, Methotrexate
4. IVIG
5. others: Cyclophosphamide, Dapsone, Plasmapheresis

Question 3

characteristic “tombstone row” of basal keratinocytes underneath the bulla on histopathology

Answer 3

phemphigus vulgaris

Question 4

MC in Older adults

Pruritic urticarial plaques and tense, large blisters. Oral mucous membrane erosions.

Subepidermal blisters with eosinophils and other inflammatory cells.

Direct immunofluorescence (IF) shows C3 and immunoglobulin (Ig) G at epidermal basement membrane zone of perilesional skin.

Indirect IF shows IgG anti-basement membrane autoantibodies in the serum.

Autoantigens BP180 and BP230 are proteins of the keratinocyte

Answer 4

BULLOUS PEMPHIGOID

Question 5

tx of bullos pemphigoid

Answer 5

1. corticosteroid : Oral prednisone, Clobetasol proprionate cream 0.05%
2. immunosupresive agents: Methotrexate, azathioprine and mycophenolate mofetil
3. modulators of antibody levels:
   IVIG, plasmapheresis
4. B-cell depletion : rituximab.
5. others:
   Dapsone and sulfapyridine
   Tetracycline and nicotinamide
   Erythromycin and nicotinamide or tetracycline alone.

Question 6

occurs in older women

characterized by evanescent vesicles that rupture quickly, leaving behind erosions and ulcers, on the mucous membranes, especially the conjunctiva

Circulating autoantibodies target the hemidesmosomal protein BP180, target C-terminal domains.

Answer 6

Cicatricial pemphigoid (“benign mucosal pemphigoid”)

previously been called ocular pem- phigus,

Question 7

tx for Cicatricial pemphigoid

Answer 7

1. cyclophosphamide + corticosteroid
2. Topical sucralfate suspension
3. dapsone, MMF, methotrexate and azathioprine

Question 8

Rare, autoimmune sub-epidermal bullous disease due to immunoglobulin G autoantibodies to type VII collagen

Skin fragility, sub-epidermal blisters, residual scarring, and milia formation

assoc. with IBD

Answer 8

EPIDERMOLYSIS BULLOSA ACQUISITA

Question 9

tx for EPIDERMOLYSIS BULLOSA ACQUISITA

Answer 9

responds poorly to treatment

systemic glucocorticoids, azathioprine, methotrexate and cyclophosphamide

oDapsone
oColchicine
oPlasmapheresis
oInfliximab
oCD 20 monoclonal antibody: Rituximab

Question 10

Intensely itchy, chronic papulovesicular eruption distributed symmetrically on extensor surfaces

Histologically: dermal papillary collections of neutrophils (microabscesses)

Diagnostic: Granular immunoglobulin (Ig) A deposits in normal-appearing skin

Dominant Autoantigens: Epidermal transglutaminase (eTG)

Most, if not all, patients have an associated gluten-sensitive enteropathy

Responds rapidly to dapsone therapy and strict adherence to a gluten-free diet

Answer 10

Dermatitis herpetiformis (DH)

Question 11

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

Bullae are often arranged in rosettes or an annular array, the so-called string of pearls configuration

Answer 11

Chronic bullous disease of childhood (CBDC)