[ XXXX --a therapeutic problem].

@entity1 @entity3217 @entity2681 @entity1219 @entity786 @entity619 @entity272 @entity13592
@entity13592 is an autoimmune subepidermal blistering disease of the skin and mucous membranes. We report on eight @entity1 in whom the clinical diagnosis was confirmed by direct immunofluorescence. The @entity1 ' age averaged 69 years; seven were female and one male. Initial symptoms were @entity2681 of the oral mucous membranes in four @entity1 , @entity272 in three @entity1 , and in one @entity1 an ocular involvement was the first manifestation of the disease. Indirect immunofluorescence on @entity1219 -split @entity1 skin revealed circulating IgG antibodies binding to the roof of the artificial blister in three @entity1 . Immunoblotting of epidermal and dermal extracts disclosed binding of IgG antibodies of one of these @entity1 to an epidermal 230-kD protein, whereas IgA-antibodies showed no specific binding. In four of five @entity1 with a strong ocular involvement IgA deposits were found by direct immunofluorescence. These studies were done on biopsies of perilesional skin or oral mucous membranes, and they were positive in one @entity1 even before the first @entity3217 appeared. Therefore, finding of IgA deposits by direct immunofluorescence may be taken as a prognostic criterion allowing selection of the proper treatment. We treated six @entity1 with a @entity619 - @entity786 pulse therapy, while two @entity1 received dapsone orally. The involvement of skin and oral mucous membrane responded well to both regimens, whereas the @entity3217 were progressive, except in one @entity1 . On the basis of our eight cases, we discuss both options and limitations in the treatment of cicatricial pemphigoid and review new aspects of the pathogenesis of this disease.
