JUVENILE XANTHOGRANULOMA WITH CENTRAL-NERVOUS-SYSTEM INVOLVEMENT

An 18-year-old man has been followed in our department since age 10 ye ars when he began to develop numerous yellow, papular lesions on the s calp, face, neck, trunk, and upper extremities. The lesions enlarged s lowly to form yellow-brown nodules measuring up to 6 cm. During this p eriod five of the nodules and two small papules were excised. Histopat hology was consistent with juvenile xanthograuloma (JXG). Recently, th e patient complained of significant loss of memory, and a computed tom ography scan was performed showing several cerebral and cerebellar les ions. The cutaneous lesions in our patient were almost identical to th ose described elsewhere as being characteristic of progressive nodular histiocytoma. The many clinical and histopathologic similarities betw een lesions of progressive nodular histiocytoma and JXG suggest that t hey may represent a continuum rather than two distinct disease process es. Given the fact that there are no histopathologic differences with JXG, as well as the wide range of clinical lesions that JXG may adopt, there is no reason to separate the entities. Although JXG lesions are usually believed to be benign and self-healing, large nodular forms c an be associated with visceral lesions. Only four previous cases of cu taneous JXG with central nervous system involvement were found in a re view of the literature.