Xanthoma disséminatum sur peau noire: retard diagnostique et difficulté thérapeutique d’un cas d’évolution fatale en Afrique subsaharienne

Medical image Xanthoma disseminatum is a proliferative non-Langerhans histiocytosis first described by Montgomery in 1938. We here report the case of a 8-year-old Senegalese boy having skin type VI who died. In 2015 this boy presented with slowly progressive dermatosis, which had occurred at the age of 7 years. Dermatological examination showed diffuse xanthomatous orange papules and nodules on the trunk, axillary and inguinal folds, scrotum, neck and face. Extradermatological examination was normal. Histopathological examination of a skin biopsy showed dermal infiltrate composed of histiocytes and multinucleated giant Touton cells. The diagnosis of non-Langerhans histiocytosis was retained, subject to immunohistochemical analyses which were not availabe. Immunohistochemical analyses were too expensive in the absence of health insurance (our patient was from a low socio-economic background). Tests measuring the amount of lipids were normal. At the age of 11 years, diabetes insipidus and systemic involvement of mediastino-pulmonary and abdominal and pelvic organs supported the diagnosis of xanthoma disseminatum. Mediastino-pulmonary disease was complicated by pneumonitis with dyspnoea which didn´t regress on prednisone treatment (1mg/kg/day). The patient underwent surgical resection of a massive mediastinal mass that compressed the organs. Five months later (in 2019), the patient died due to respiratory distress. The difficulty of diagnosing non-Langerhans histiocytosis in our context is largely related to the inaccessibility of immunohistochemistry. Indeed, if CD68 positive and CD1a negative labeling cells had been early detected, fairly early diagnosis would be made. Thus, we could have offered treatments to improve the quality of child´s life.