Summary

Sezary Syndrome (SS) represents the leukemic variant of the cutanaeus T-cell lymphoma (CTCL). It is the most aggressive form cu cutaneous lymphoma with an annual at 1/10,000 and comprises 3% of all cases of CTCL. The onset of the disease often occurs with rapid onset of erythemato-squamous plaques accompanied by severe itching that mimic eczema, psoriasis or drug skin reactions. We present the clinical course of 65-year-old patient diagnosed with Sezary syndrome and followed for seven years. He was admitted for a skin eruption composed of large erythematous plaques with fine scale, highly pruritic that involved the head, the trunk and the root of the limbs. We noted also associated fissured plaques of the palms and soles, onycholysis, axillar and inguinal polyadenopathy, physical asthenia and inappetence. Repeated previous biopsies reported nonspecific histopathological changes. A new cutaneous biopsy revealed an inflammatory lympho-histiocytic infiltrate in the papillary and the reticular dermis disposed in dense perivascular aggregations with ascending solitary lymphocytes in the basal layer and the formation of rare intraepidermic nests. Lymphocytes observed at the interstitial level were small and medium in size, with enlarged, polyhedral and irregularly contoured nuclei. A peripheral blood flow cytometry was performed which detected the presence of a lymphocyte clone with identical phenotype to that found in the cutaneous biopsies. A lymph node biopsy, revealed a disrupted microscopic architecture, proliferation of medium to large-sized cells with irregular nuclear contours, hyperchromatic or vesicular nuclei, prominent nucleoli, and atypical mitoses. However, residual lymphoid follicles could be occasionally observed. After the diagnosis, the patient was treated with different agents according to the status like chemotherapy, adjuvant treatment, retinoids. Local treatment consisted in topical steroids, UVB nb and PUVA. Due to multiple organ involvement, the patient died seven years after the diagnosis of SS was performed. This aspect is highly particular for this case, according to the literature data the medium life expectancy after the diagnosis of SS is about 2 years.