PARANEOPLASTIC PEMPHIGUS - A CASE WITH HIGH-TITER OF CIRCULATING ANTIBASEMENT MEMBRANE ZONE AUTOANTIBODIES

Multiple flaccid bullae, erosions, and tense bullae developed in a 67- year-old man with chronic lymphocytic leukemia. A biopsy specimen reve aled features of typical pemphigus vulgaris (suprabasilar clefting wit h acantholysis), paraneoplastic pemphigus (necrotic keratinocytes and hydropic degeneration in addition to acantholysis), and bullous pemphi goid (subepidermal separation and epidermal regeneration). Direct and indirect immunofluorescence studies revealed deposits of immunoglobuli ns and C3 within the intercellular substance and basement membrane zon e in some specimens. Immunoprecipitation of serum revealed four polype ptides with molecular weights of 250, 230, 210, and 190 kd.