ESSENTIALS OF DIAGNOSIS
Relapsing crops of bullae, often fragile and leading to erosions.
Often preceded by mucous membrane bullae, erosions, and ulcerations.
Superficial detachment of the skin after pressure or trauma variably present (Nikolsky sign).
Acantholysis on biopsy.
Immunofluorescence studies and serum ELISA for pathogenic antibodies are confirmatory.
Pemphigus is an uncommon intraepidermal blistering disease occurring on skin and mucous membranes. It is caused by autoantibodies to adhesion molecules expressed in the skin and mucous membranes. The cause is unknown, and in the preantibiotic, presteroid era, the condition was usually fatal within 5 years. The bullae appear spontaneously and are tender and painful when they rupture. Drug-induced pemphigus from penicillamine, captopril, and others has been reported. There are several forms of pemphigus: pemphigus vulgaris and its variant, pemphigus vegetans; and the more superficially blistering pemphigus foliaceus and its variant, pemphigus erythematosus. All forms may occur at any age, but most present in middle age. The vulgaris form begins in the mouth in over 50% of cases. The foliaceus form is especially apt to be associated with other autoimmune diseases, or it may be drug-induced. Paraneoplastic pemphigus, a unique form of the disorder, is associated with numerous types of benign and malignant neoplasms but most frequently non-Hodgkin lymphoma.
Pemphigus is characterized by an insidious onset of flaccid bullae, crusts, and erosions in crops or waves (Figure 6–28). In pemphigus vulgaris, lesions often appear first on the oral mucous membranes. These rapidly become erosive. The scalp is another site of early involvement. Rubbing a cotton swab or finger laterally on the surface of uninvolved skin may cause easy separation of the epidermis (Nikolsky sign). Downward pressure on a fresh bulla may cause lateral spread (Asboe-Hansen sign). Pemphigus vegetans presents as erosive vegetating plaques, most often in intertriginous areas. Pemphigus foliaceus is a superficial form of pemphigus where cutaneous lesions present as flaccid bullae that quickly evolve into superficial erosions and thin pink plaques with overlying scale. Mucosal lesions are rare in pemphigus foliaceus. Pemphigus erythematosus has overlapping features of pemphigus foliaceus and lupus erythematosus. It presents with flaccid bullae that develop overlying scale and crust in a photodistributed area. Again, mucosal lesions are rare. Paraneoplastic pemphigus is histologically and immunologically distinct from other forms of the disease. While clinical lesions may not be distinct from pemphigus vulgaris, oral lesions predominate and cutaneous erythematous plaques resembling erythema multiforme are characteristic. Survival rates are low because of the underlying malignancy.
Pemphigus vulgaris on the back with crusted and intact bullae. (Used, with permission, from Eric Kraus, MD, in Usatine RP, Smith MA, Mayeaux EJ Jr, Chumley H. The Color Atlas of Family Medicine, 2nd ed. McGraw-Hill, 2013.)