Sézary-like syndrome in a 10-year-old girl with serologic evidence of human T-cell lymphotropic virus type I infection.

A 10-year-old girl with a Sézary-like syndrome is described. At age 6 months, her skin involvement began as psoriatic plaques. Generalized erythema, lymphadenopathy, and pruritus developed into erythroderma. Her general condition has been good in spite of severe pruritus and erythroderma. Atypical cells were found in the peripheral blood in numbers of 2500/mm3 and were present in the dermis of a skin biopsy specimen. When examined by electron microscopy, these cells showed lobulated or indented nuclei and were similar to Sézary cells. The patient showed a high titer (1:1280) of antihuman T-cell lymphotropic virus type I antibody. Her mother was also positive with a titer of 1:40 and seems to be a healthy carrier.