Paraneoplastic pemphigus: a pustulosis variant associated with chronic lymphoid leukemia

Background. Paraneoplastic pemphigus is an autoimmune disease of the skin a nd mucosa described in 1990. The condition is generally associated with lym phoma or chronic lymphoid leukemia. Lesions are often misleading, masquerad ing as polymorphous erythema or lichen. We report a case of paraneoplastic pemphigus with pustulous skin lesions. Case report. A 52-year-old man developed over a few weeks time erosive lesi ons of the oral cavity and lips associated with papulous skin lesions. Seco ndarily, large-sized pustules, sometimes a hypopion, were observed associat ed with bullae. The diagnosis of paraneoplastic pemphigus was confirmed by direct immunofluorescence that evidenced IgG deposits within the keratinocy tes and along the basal membrane and by indirect immunofluorescence on rat bladder that evidenced circulating antibodies. This paraneoplastic pemphigu s was the inaugural sign of chronic lymphoid leukemia. Discussion. Skin lesions described in paraneoplastic pemphigus include: ero sion, vesicles, bullae, and psoriasiform, lichen-like, plate-like or vegeta tive formations. To our knowledge, this is the first report of a pustulous Form; clinically similar to Hallopeau pustulous pemphigus.