Infantile Xanthogranuloma

Abstract

Juvenile Xanthogranuloma is a granulomatous histiocytosis, which develops benignly with an intracellular storage of lipidic material and without any alteration of lipid metabolism. Although it usually appears at a very early age (1 to 2 year old children), it may also develop later on in children aged 4 to 12, or even in adults or people over 50. Regression is spontaneous and constant, and generally takes place over the course of a few years. Medical control enables us to make a morpho evolutionary classification of cases: the form may be papular, nodular or mixed; eruptive with a great number of elements, with few lesions or only one. The histological study of lesions at different stages of development records the steps through which the process, at first simply histiocytic, becomes granulomatous with more or less numerous giant cells, lymphocytes, eosinophils and sometimes plasmacells. Although usually cutaneous, the process may frequently also involve the eye, either alone on in association with cutaneous lesions; in these cases, diagnositc and therapeutic problems arise. The involvement of lung, bone or other organs is unusual, although even in these rare cases, recovery is spontaneous, albeit slow. The various differential diagnoses, sometimes very puzzling from the clinical viewpoint but solvable upon histological examination, are dealt with; in particular, the eruptive forms with scratch produced whealing (differentiation from a form of Urticaria pigmentosa) and the single lesion forms (differentiation from xanthomatous histiocytofibroma, juvenile melanoma, mastocytoma, angioma) are discussed. The spontaneous resolution of the disease does not raise any therapeutic problem, except when the eye is involved.