Diagnosis: Bullous Pemphigoid
A 27-year-old otherwise healthy man presented with a 2-day history of pruritic blisters that were scattered on the trunk and proximal extremities. Two weeks after admission, oral mucous membrane lesions developed, the patient complained of pain on swallowing, and the skin involvement had increased to 90% of the body surface area. Histology revealed a subepidermal blister with a superficial, perivascular infiltrate with eosinophils, lymphocytes, and neutrophils. Numerous eosinophils were present in the blister cavity, and direct immunofluorescence showed linear staining of IgG, IgA, and C3 along the basal membrane. ELISA showed the presence of IgG autoantibodies against bullous pemphigoid antigen 2 (BPAG2, 180 kd), but none against bullous pemphigoid antigen 1 (BPAG1, 230 kd), said Dr. Amanda S. Buchau of the dermatology department at Heinrich Heine University, Düsseldorf, Germany with mucosal involvement in 10%–20% of cases. The condition may result from an increased expression of anti-BP-180 autoantibodies, which are considered a marker of poor prognosis in bullous pemphigoid.
This patient was treated with methylprednisolone up to 160 mg/day for 3 weeks, along with pulse cyclophosphamide 1,000 mg, without response. His antibody titer also remained very high, so plasmapheresis was performed. Treatment with 250 mg/day of prednisone and 1 g of mycophenolate mofetil twice daily stabilized the disease, and he was given ciprofloxacin, dicloxacillin, and piperacillin to prevent infections, as well as antihistamines and topical steroids to alleviate pruritus.
Dr. Buchau presented this case at a congress on skin, rheumatism, and autoimmunity held in Abano Terme, Italy.
The otherwise healthy man presented with a 2-day history of pruritic blisters across the trunk and proximal extremities.
Histology revealed a subepidermal blister with a perivascular infiltrate with eosinophils, lymphocytes, and neutrophils. Photos courtesy Dr. Amanda S. Buchau