[Paraneoplastic pemphigus: a pustular form during chronic lymphoid leukemia]

Paraneoplastic pemphigus is an autoimmune disease of the skin and mucosa described in 1990. The condition is generally associated with lymphoma or chronic lymphoid leukemia. Lesions are often misleading, masquerading as polymorphous erythema or lichen. We report a case of paraneoplastic pemphigus with pustulous skin lesions.

A 52-year-old man developed over a few weeks time erosive lesions of the oral cavity and lips associated with papulous skin lesions. Secondarily, large-sized pustules, sometimes a hypopion, were observed associated with bullae. The diagnosis of paraneoplastic pemphigus was confirmed by direct immunofluorescence that evidenced IgG deposits within the keratinocytes and along the basal membrane and by indirect immunofluorescence on rat bladder that evidenced circulating antibodies. This paraneoplastic pemphigus was the inaugural sign of chronic lymphoid leukemia.

Skin lesions described in paraneoplastic pemphigus include: erosion, vesicles, bullae, and psoriasiform, lichen-like, plate-like or vegetative formations. To our knowledge, this is the first report of a pustulous form; clinically similar to Hallopeau pustulous pemphigus.