Schnitzler's syndrome: A female elderly case presenting intractable non-pruritic febrile urticarial rush.

Schnitzler's syndrome is an acquired autoinflammatory disease characterized by chronic urticarial rash and monoclonal gammopathy (predominantly IgM type). A 75-year-old Japanese woman complained of high fever and non-pruritic urticarial rash appearing almost every day for 3 years. Her abnormal laboratory data included leukocytosis and neutrophilia with elevated erythrocyte sedimentation rate and C-reactive protein level.

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

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.