A Rare Presentation of Erythrodermic Mycosis Fungoides

Although rare, of all the cutaneous lymphoid malignancies, cutaneous T-cell lymphomas (CTCLs) constitute 65% of all lymphomas, of which 50% are patients with mycosis fungoides (MF). The erythrodermic variant of MF, a malignancy of mature, skin homing, clonal T lymphocytes, usually presents in mid to late adulthood. We present a man in his late 30s with intractable progressive erythroderma of 18 months’ duration, patchy alopecia, palmoplantar keratoderma, mucosal thickening, hyperpigmentation, and intense itching as a case of erythrodermic MF. There was no systemic involvement. Diagnosis was confirmed by biopsies from multiple sites and immunohistochemistry. He was categorized with stage IIIA MF according to the International Society for Cutaneous Lymphomas/European Organization of Research and Treatment of Cancer (ISCL/EORTC) revised classification system. Psoralen plus UVA therapy and low-dose methotrexate resulted in clearance of lesions; regular follow-up visits were conducted to monitor progression to Sézary syndrome (SS). Progression to SS has to be monitored regularly in these patients.