Pilotropic CD30 positive lymphoma.

Background. We report an unusual case of cutaneous CD30-positive lymphoma w ith pilar tropism and circulating Sezary cells which had a rapidly fatal co urse. Case report. A 78-year-old man presented erythematous infiltration of the f ace, a pruriginous eruption on the trunk and proximal portions of the limbs with small erythematopurpuric follicular papulae, and node enlargement in the inguinal and axillary areas. The rest of the clinical examination was n ormal. Circulating Sezary cells were found in significant numbers on two di fferent blood smears. Histologic and immunohistochemistry examination of a skin biopsy evidenced medium to large sized lymphoid cell infiltration in a perifollicular localization. A few small cells penetrated the pilar appara tus. There was no follicular mucinosis. The tumoral cells expressed CD2, CD 3, CD4 and 75 p. 100 were positive for CD30. Node aspiration showed lymphom atous cells and CD3+ and CD30+ lymphomatous infiltration was found on marro w smears. A T clone was evidenced both in blood and bone marrow leading to the diagnosis of pilotropic CD30-positive lymphoma. Chlorambucil and predni sone were given. The patient died 5 months later. Discussion. The cytology findings suggest medium to large cell pleomorphic lymphoma. The circulating Sezary cells, the pilotropic eruption, and the ra pidly fatal outcome suggest transformation of a Sezary syndrome into CD30-p ositive large cell lymphoma which has been described in fungoid mycosis.