LEUKEMIC COURSE OF MYCOSIS-FUNGOIDES REVE ALED BY REACTIVE HEMOPHAGOCYTIC SYNDROME

Background. Mycosis fungoides can mimic pigmented purpuric dermatitis. We report such a case which progressed to peripheral T-cell lymphoma; progression was revealed by reactive hemophagocytic syndrome (RHS). C ase report. A 65-year-old male patient was hospitalized for a pigmente d and purpuric eruption. The skin lesions appeared 2 years earlier and al that time biopsy had shown pigmented and purpuric dermatitis. One month before hospitalization, general signs appeared. On admission, he had papular and purpuric rash, mainly on the trunk, hepatosplenomegra ly, enlarged axillar and inguinal lymph nodes, and fever at 38.2 degre es. A skin biopsy showed histologic changes typical of mycosis fungoid es. Hr also had bicytopenia, hepatitis, and increased triglyceride and ferritin levels suggesting RHS which was proved by means of bone marr ow biopsy. These tests also evidenced peripheral T-cell lymphoma. The patient was treated with two courses of chemotherapy (CHOP) but the di sease progressed and he deceased. Discussion. Mycosis fungoides can oc casionally begin with an eruption very closely resembling pigmented pu rpuric dermatitis. Therefore, repeated biopsies should be done in case of widespread permanent pigmented purpuric dermatilis of no apparent origin. RHS is a life-threatening disease. The diagnosis should be sus pected in any cytopenic patient with fever, increased triglyceride lev els and abnormal liver tests. A starch for an etiology must then be un dertaken as prompt treatment is needed.