Trichothiodystrophy without associated neuroectodermal defects

We report a case of adult T-cell leukaemia/lymphoma (ATLL) with prurigo as a prodromal skin manifestation. The patient was a 52-year-old woman with a 2-year history of a fluctuating skin condition consisting of pruritic papules and pigmentation on her lower legs and right buttock. Prurigo was diagnosed at her first visit in October 1995, and she was found to be seropositive for human T-cell lymphotropic virus type 1 (HTLV-1). Two years later, the skin lesions had spread over the patient's forearms and dorsa of the hands, and abnormal lymphoid cells had appeared in her peripheral blood. Southern blot analysis revealed monoclonal integration of HTLV-1 provirus in the peripheral blood, but not in a skin lesion. Based on these results, a diagnosis of overt ATLL of the smouldering type was made. The findings in this case indicate that in healthy HTLV-1 seropositive carriers, prurigo requires careful follow-up as a cutaneous prodrome of ATLL.