Blistering diseases

Chapter 9


Blistering diseases




Subcorneal vesiculobullous disorders



Pemphigus foliaceus




Pemphigus foliaceus is a subcorneal vesiculobullous disorder caused by autoantibodies directed at an intercellular keratinocyte adhesion protein, desmoglein 1 (160 kD). The disease usually presents with superficial crusted erosions upon the face and upper trunk. The superficial nature of the blisters makes them fragile and most patients lack intact bullae.


The presence of a subcorneal blister, with acantholytic cells and scattered eosinophils, is highly suggestive of pemphigus foliaceus. Direct immunofluorescence (DIF) is diagnostic and demonstrates intercellular IgG and C3 deposition, primarily confined to the upper half of the epidermis. The split occurs in the granular layer, as in staphylococcal scalded-skin syndrome. Pemphigus foliaceus may demonstrate neutrophils within the vesicle, making distinction from bullous impetigo difficult. A tissue Gram stain may be helpful, but the presence of an impetiginized crust does not rule out pemphigus foliaceus.


Pemphigus erythematosus blends the immunohistologic findings of pemphigus foliaceus with lupus erythematosus. Patients often have a positive serum antinuclear antibody. Immunofluorescence yields both an intercellular deposition of immunoreactants and a “lupus band” of immunoreactants along the basement membrane zone.




Subcorneal pustular dermatosis (Sneddon–Wilkinson disease)




Cases of subcorneal pustular dermatosis with intercellular deposition of IgA have been reclassified as IgA pemphigus. Subcorneal pustular dermatosis may represent a subclass of pustular psoriasis, although mitotic figures within the underlying epidermis, common to psoriasis, are not identified in subcorneal pustular dermatosis. The classic patient is an older woman with annular or polycyclic lesions of the trunk or groin with pustules at the periphery.




Intraepidermal vesiculobullous disorders



Pemphigus vulgaris





Pemphigus vulgaris is an intraepidermal vesiculobullous disorder caused by autoantibodies directed at an intercellular keratinocyte adhesion protein, desmoglein 3 (130 kD). The disease presents with erosions of the skin and mucosa. Often the disease begins in the posterior oropharynx weeks before cutaneous lesions are noted. Most patients lack intact bullae. Erythematous skin shears away easily when lateral pressure is applied (Nikolsky sign).


Clefting above the basal layer, with acantholysis of the remaining basilar keratinocytes, leads to a visual impression likened to “rows of tombstones” sitting upon the dermal papillae. Tracking of the blistering process down adnexal structures is often demonstrated. Direct immunofluorescence is diagnostic and demonstrates intercellular IgG and C3 deposition, primarily confined to the lower half of the epidermis.


Pemphigus vegetans is a related disorder which demonstrates vegetative cutaneous lesions with epidermal hyperplasia and lesser vesiculation. Suprabasilar crypts containing eosinophils may be identified within the acanthotic epidermis in many cases of pemphigus vegetans.




Familial benign chronic pemphigus (Hailey–Hailey disease)





Hailey–Hailey disease is an autosomal-dominant, inherited disease caused by mutations in the ATP2C1 gene. This gene encodes for a portion of a calcium pump essential for proper keratinocyte differentiation and adhesion. Skin of the intertriginous areas is most prominently affected and yields a clinical appearance likened to “wet tissue paper.” Acantholysis at all levels of the epidermis yields the histologic appearance of a “dilapidated brick wall.” While the disease itself is not immunologically mediated, superinfection of macerated skin by bacteria or yeast may engender an underlying inflammatory infiltrate within the superficial dermis.




Keratosis follicularis (Darier’s disease)



Apr 26, 2016 | Posted by in Dermatology | Comments Off on Blistering diseases
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