2022 Volume 21 Issue 3 Pages 200-205
A 61-year-old man was referred to our hospital because of erosions on his nasal and oral mucosa and blisters on his trunk and extremities. Histopathological examination revealed subepidermal blister formation. Direct immunofluorescence showed linear deposition of IgG and C3 on the basement membrane zone. Indirect immunofluorescence using 1 M NaCl split skin showed the presence of IgG autoantibodies reactive to the dermal side. Western blot analysis using dermal extract showed autoantibody reactive to 290 kDa protein. ELISA showed the presence of autoantibodies for type VII collagen. Therefore, we diagnosed the patient as mucous membrane pemphigoid (MMP) with antibodies against type VII collagen. Although his cutaneous lesions were easily improved by oral prednisolone, mucous lesions were refractory, which finally improved by additional treatment with plasmapheresis, intravenous immunoglobulin, diaminodiphenylsulfone,azathioprine, tetracycline, and niacinamide. We speculate that epitopes of autoantibodies against type VII collagen are different in MMP from those in epidermolysis bullosa acquisita, which mainly show cutaneous lesions. Skin Research, 21 : 200-205, 2022
