Anti-p200 pemphigoid

Anti-p200 pemphigoid is arare autoimmune blistering disease. It belongs to the group of pemphigoid diseases and was first described in 1996. The diagnostic gold standard is the combination of (1)linear deposits of immunoreactants at the dermal epidermal junction by direct immunofluorescence microscopy of aperilesional skin biopsy, (2)detection of circulating autoantibodies binding to the dermal side (blister floor) of human salt split skin by indirect immunofluorescence microscopy, and reactivity with a200kDa protein (p200) in extract of human dermis by immunoblotting. In 2009, laminin 1 was described as an additional target antigen in 90% of anti-p200 pemphigoid patients. Since ex vivo and in vivo studies have shown no direct pathogenic relevance for laminin 1 antibodies and the preadsorption of patient sera against laminin 1 does not reduce their reactivity with p200, the molecular identity of p200 still remains to be elucidated. The clinical phenotype of the disease is heterogeneous; in most cases, however, it resembles bullous pemphigoid. Anti-p200 patients are younger and skin lesions more often appear on palms of the hands and soles of the feet than in bullous pemphigoid. Therapy consists of topical and systemic corticosteroids. In addition, the use of daspone and immunosuppressants has been reported.