Histiocitosis X (HX) represents a group of disorders due to proliferation of abnormal histiocytes exhibiting immunophenotypical and ultrastructural features similar to Langerhans cells. HX can become manifest as an isolated lesion or may affect several organs at once. In particular, cutaneous involvement may represent the earliest sign of the disease, but is only rarely observed as the sole clinical manifestation. Congenital cases are also very rare. We report a case of congenital HX with skin lesions, without any sign of systemic involvement. At birth, the female infant was noted to have a papular eruption involving the scalp, forehead and extremities, which later spread to the trunk. Hematological, roentgenographic and echographic examinations were normal. Biopsy specimens of 2 lesions revealed a dense dermal infiltrate of large histiocytes, with irregularly indented nuclei; these cells showed immunohistochemical and ultrastructural features typical of Langerhans cells. After initial treatment with prednisone alone, a systemic chemotherapy (vinblastine + cyclosphamide) was started to prevent cutaneous recurrences and/or extracutaneous dissemination, and stopped after 11 months. Currently, after a three-year follow-up period, the little patient shows no evidence of disease.
|Translated title of the contribution||Congenital histiocytosis X with 'pure' cutaneous involvement. Report of a case|
|Number of pages||5|
|Journal||Giornale Italiano di Dermatologia e Venereologia|
|Publication status||Published - 1993|
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