Autoimmune bullous diseases are uncommon but have dramatic presentations. Bullous pemphigoid (BP) results from autoantibodies to the epidermal basement membrane and creates tense bullae, frequently located on the proximal extremities. In pemphigus vulgaris (PV), the autoantibodies are directed against the epidermal keratinocytes. This results in flaccid bullae (more superficial bullae that easily slough off to form erosions). The delicate bullae and subsequent erosions of PV commonly present in the pharynx, scalp, and trunk. Paraneoplastic pemphigus presents with severe oral ulcerations (like SJS/TEN; see related item) and resolves with treatment of the associated malignancy.
Management and Disposition
Considering an autoimmune bullous disease in the differential is the 1st step. Admission with early dermatologic consultation for histologic and immunofluorescent studies should be considered. Systemic corticosteroids and immunosuppressant therapy are required for control. Patients with significant BSA involvement should be treated in a burn unit.
Oral erosions and ulcerations should always raise the suspicion of autoimmune bullous diseases.
The high morbidity and mortality of this disease is now significantly lower due to modern steroid-sparing immunosuppressants and wound care.
Always consider medication history and signs of underlying malignancies when evaluating a patient with extensive bullae or erosions.
Bullous Pemphigoid. Tense blister formation among confluent erosions and plaques. (Photo contributor: Ben Heavrin, MD.)
Bullous Pemphigoid. Tense bullae on in the distal extremities are a common presentation of BP. (Photo contributor: Selim Suner, MD, MS.)
Pemphigus Vulgaris. Multiple flaccid bullae now de-roofed and at risk for secondary infection. (Photo contributor: J. Matthew Hardin, MD.)