A case of primary cutaneous aggressive epidermotropic cd8+ cytotoxic t-cell lymphoma misdiagnosed as febrile ulceronecrotic mucha-habermann disease

Abstract

Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma (pCAE-CD8+CTCL) is a rapidly progressing cutaneous lymphoma characterized by malignant proliferation of epidermotropic CD8+ T cells. Febrile ulceronecrotic Mucha-Habermann Disease (FUMHD) is an inflammatory dermatosis of unknown aetiology manifested by ulcerative and necrotic lesions accompanied by fever. These diseases are both extremely rare and share some common features. We report here a case of pCAECD8+ CTCL initially misdiagnosed as FUMHD.