CombinedCase of Anti-laminin γ1 Pemphigoidand Anti-laminin 332 Mucous Membrane Pemphigoid

Abstract

A 77-year-oldman was referredto us because of a one-week history of erosions on the lips, oral cavity, andpenis, andnumerous erythemas andblisters on the trunk andextremities. Chemiluminescent enzyme immunoassays did not detect anti-BP180-NC16A domain autoantibodies or anti-desmoglein 1 and 3 autoantibodies. Histopathological examination showed subepidermal bullae with infiltration of eosinophils andneutrophils. Direct immunofluorescence detected linear deposition of C3 at the basement membrane zone (BMZ). Indirect immunofluorescence using normal skin as substrate showed IgG anti-BMZ antibodies, which reacted with the dermal side of 1M NaCl-split skin. Immunoblotting with normal human dermal extract detected 200 kDa laminin γ1 (p200), andimmunoblotting with laminin 332 recombinant proteins detected165kDa laminin α3. We made a diagnosis of coexistence of anti-laminin γ1 pemphigoidandanti-laminin 332 mucous membrane pemphigoid. Intravenous administration of prednisolone led to quick improvement of the mucocutaneous lesions. Our findings suggest the need to establish more convenient strategies for the diagnosis of both anti-laminin γ1 pemphigoidanti-laminin 332 mucous membrane pemphigoidfor differential diagnosis and appropriate treatments for these diseases. Skin Research, 21 : 126-132, 2022