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Niemann-Pick disease and juvenile xanthogranuloma. Are they related?
W S Wood1, J E Dimmick, C L Dolman
1Department of Pathology, University of British Columbia, Vancouver General Hospital, Canada.
The American Journal of Dermatopathology
|October 1, 1987
Abstract:
Niemann-Pick disease is a rare autosomal recessive lipidosis with accumulation of sphingomyelin in multiple organs due to sphingomyelinase deficiency. Cutaneous lesions are uncommon. We describe a case of Niemann-Pick disease with multiple cutaneous papules and nodules resembling juvenile xanthogranuloma.