Cicatricial pemphigoid with autoantibodies against the laminin 5 gamma 2 subunit.

ABSTRACT

Cicatricial pemphigoid (CP) is an autoimmune bullous disease accompanied by mucosal lesions. The majority of patients with CP have autoantibodies against BP180. Anti-laminin 5 (epiligrin) CP is relatively rare. It is known that, in most cases, circulating autoantibodies against laminin 5 in these patients recognize the alpha3 and/or beta3 subunits of this molecule. Here we report a case of anti-laminin 5 CP, which showed IgG autoantibodies against the gamma2 subunit of laminin 5 alone. A 50-year-old woman suffered from skin blistering on the trunk and extremities and severe mucosal lesions in the eyes, oral cavities and laryngopharynx. Despite potent systemic steroids, the mucosal lesions and some parts of the skin lesions persisted. Salazosulfapyridine was of value in controlling the laryngopharyngeal lesions and persistent cutaneous blistering, and cyclophosphamide had definite effects especially on ocular lesions. Anti-laminin 5 autoantibodies became undetectable in serum from the patient after the disease was controlled.