PARANEOPLASTIC PEMPHIGUS - REVIEW OF THE LITERATURE, ABOUT ONE CASE ASSOCIATED WITH CHRONIC LYMPHOID LEUKEMIA

In 1990, Anhalt et al described a newly autoimmune bullous disease: pa raneoplastic pemphigus, in five patients. It was characterized by a di stinct set of circulating autoantibodies from those in the sera of pat ients with pemphigus vulgaris and superficial pemphigus. We report a 7 1 year-old man with chronic lymphocytic leukemia of 4 years duration w ho developed a severe mucocutaneous eruption with clinical and immunof luorescence findings of pemphigus vulgaris evolving into an oral bullo us lichen planus presentation. Evaluation of his serum confirmed the p resence of autoantidodies specific for paraneoplastic pemphigus by ind irect immunofluorescence on rat-bladder and immunoprecipitation. Subse quently, additional cases have been reported in the literature. All oc cured in patients with various neoplastic conditions. These patients p resent with polymorphous skin lesions and severe erosive and disease. Histologic examination shows interface dermatitis and keratinocyte nec rosis in addition to acantolysis. Direct immunofluorescence may reveal deposition of immunoglobulin and/or complement at the basement membra ne as well as deposition on epithelial cell surfaces. Circulating IgG anti-cell-surface antibodies are detectable with both stratified and s tratified epithelial as substrates. These antibodies immunoprecipitate a complex of four desmosomal proteins. including desmoplakin I (250 k Du), the bullous pemphigoid antigen (230 kDa), desmoplakin II (210 kDa ), and a 190 kDa antigen.