MELANODERMA AS AN EARLY CLINICAL MANIFESTATION OF SÉZARY SYNDROME

Primary cutaneous lymphomas are a heterogeneous group of hematologic malignancies originating from the T-cells or B-cells residing within the human skin. More than two-thirds (70-85%) of cases are diagnosed as cutaneous T-cell lymphomas (CTCL) with Mycosis fungoides and Sézary syndrome being the most notable. CTCL have an annual incidence of 10 cases per 1 million, with a predominance to males over the age of 50 (male-to-female ratio of 2:1). Here, we present a case of a 54-year-old female who presented with persistent and generalized hyperpigmentation, later evolving into Sézary syndrome. Initially, the patient was reffered to the University Clinic for Dermatology in Skopje, due to a five-month medical history of persistent pruritus and hyperpigmentation. Based on the clinical presentation and skin biopsy findings consistant with non-specific dermatitis, she was diagnosed as having atopic dermatitis and underwent treatment with topical steroids and emollients. However, the clinical presentation got worsen in the following six months, and evolved into generalized melanodermia with ulcerated tumor growths. In order to conclude the final diagnosis, a biopsy of the skin lesions was performed with histopathological findings consistent with Sézary syndrome. Following this diagnosis, the patient was referred to the University Clinic for Hematology in Skopje for further treatment.