Paraneoplastic pemphigus as the initial presentation of chronic lymphocytic leukemia

The case history of a 61-year-old male patient is described, who presented with severe stomatitis, conjunctivitis and leukocytosis. The diagnosis chro nic lymphocytic leukemia (CLL) stage A (0) was made, for which no treatment was necessary. Progression of stomatitis and conjunctivitis and erythosqua mous skin lesions with bullae and vesiculae formation developed. Under the diagnosis of bullous pemphigoid the patient was treated with corticosteroid s. The histologic and immunofluorescence examination of a skin biopsy was c ompatible with this diagnosis, and antibodies to skin could not be detected in a first serum sample. Pseudomonas was cultured from all lesions, the co rticosteroids were stopped and antibiotic treatment was started, without cl ear effect. Because of progression of skin lesions and debilitation , the p atient finally declined all treatment and died five weeks after admission. Post-mortem examination showed enlarged lymphnodes in the cervical, aortal en iliacal areas, with histology confirming the diagnosis of CLL. Indirect immunofluorescence with the second serum sample showed auto-antibodies in h igh titer directed against the intercellular epithelial substance. Immunobl ot studies showed binding with the classic target antigens in paraneoplasti c pemphigus. Re-examination of the histologic skin specimen and the result of direct immunofluorescence were in retrospect compatible with the diagnos is of paraneoplastic pemphigus.