Mycosis fungoides and Sezary syndrome against a human immunodeficiency virus-positive background: case report

Abstract

Background: Mycosis fungoides (MF) and Sezary syndrome (SS) are forms of primary cutaneous T-cell lymphoma. They are relatively rare diseases, infrequent in Europe, Asia, and Africa. In Rwanda, they remain very rare. Their etiopathogenesis has long been obscure and poorly understood. Recent studies, however, have confirmed that they are of viral origin, specifically human T-cell lymphotropic virus type I (HTLV-I) and human immunodeficiency virus (HIV). Their incidence is low: about three new cases per year per million inhabitants. Individuals affected are usually aged between 40 and 60 years and of male gender. The incidence of SS is less than one new case per year per million inhabitants.

Methods: A new case of MF/SS syndrome against a HIV-positive background is described and a literature review is performed.

Results: Clinically, MF is characterized at onset by pruriginous, disseminated, erythematous, scaly plaques, localized on the skin, which gradually become infiltrative. In advanced stages, the nodular lesions can become ulcerated and Sezary cells are detected in the peripheral blood. Our male Rwandan MF/SS/HIV-positive patient presented with pruriginous, erythematous, scaly lesions, which had developed over 4 years and were now infiltrative, together with nodular lesions of various sizes, localized on the face, scalp, and palmar and plantar regions. All of these clinical signs, the anatomopathologic examination, retroviral serology, and testing of peripheral blood confirmed the diagnosis of MF/SS against a background of immunodepression.

Conclusions: MF and SS are relatively rare cutaneous lymphomas, but have been diagnosed in Rwanda against a clinical picture of immunodepression. Their etiology seems to be viral in origin. MF and SS are difficult diseases to diagnose, with confirmation only at advanced stages. No tests exist to aid and confirm the diagnosis in the initial stages of the diseases. Further studies are urgently needed on these diseases.