An unusual presentation of mucous membrane pemphigoid

A 68-year-old woman presented with recurrent mucocutaneous blisters of 6 years' duration and progressive loss of scalp hair of 2 years' duration. The disease had started as tense blisters associated with a burning sensation over the lower part of her back, gradually followed by involvement of her chest, upper part of her back, and arms. The blisters persisted for weeks before rupturing spontaneously. Four years later, she developed tense oral blisters, resulting in painful persistent erosions. At about the same time, blisters on her scalp developed, followed by erosions and hair loss. No other mucosal sites were involved. She had no symptoms of photosensitivity, joint pain, dysphagia, weight loss, or other systemic complaints. On examination, multiple tense bullae and well-defined deep erosions were seen over the hard and soft palates (Figure 1). Cutaneous examination revealed a few tense bullae on normal-looking skin over her abdomen (Figure 2 inset) and arms and areas of scarring at the site of healed lesions on her back. Her scalp had bullae of similar morphology, crusted erosions, and cicatricial alopecia at the site of previous lesions involving a large area of scalp (Figure 2). Examination of the other mucosae did not reveal any abnormality. Histologic studies with hematoxylin and eosin stain of a skin biopsy specimen revealed deroofed subepidermal bullae with dense dermal inflammatory infiltrate predominantly composed of eosinophils (Figure 3). Direct immunofluorescence of a biopsy specimen from perilesional skin revealed linear deposits of immunoglobulins M and G and of C3 at the dermoepidermal junction which was consistent with mucous membrane pemphigoid. This patient was prescribed prednisolone 30 mg daily and dapsone 100 mg daily, following which there were no new blisters. At 3 months' follow-up, previous erosions had partially healed.