Refractory anti-laminin γ1 pemphigoid with psoriasis vulgaris successfully treated by double-filtration plasmapheresis

摘要

Anti-laminin gamma1 pemphigoid, [1], formerly known as anti-p200 pemphigoid, is an autoimmune bullous disease, characterized by the presence of circulating IgG autoanti-bodies reactive with dermal side of 1M NaCl normal human split skin by indirect immunofluorescence and with 200 kDa laminin gamma1 by immunoblotting of normal human dermal extracts. The association with psoriasis is well-known [2]. Anti-laminin gamma1 pemphigoid usually shows a good response to oral corticosteroids, cyclosporine, minocycline or dap-sone [2, 3], and refractory cases are rare [3]. We report a severe case of anti-laminin gamma1 pemphigoid, unresponsive to oral corticosteroid. The patient was relatively old and also had psoriasis. Additional double-filtration plasma-pheresis (DFPP) and subsequent cyclosporine resulted in a good response of both diseases without side effects.