DOI: 10.36347/sjmcr.2020.v08i01.014

Pages: 46-48

Juvenile xanthogranuloma is a rare disease. It is a benign, auto-involutional tumor, mainly of cutaneous localization. We report 4 cases of multiple juvenile xanthogranuloma. There were 4 male children, whose average age was 1 year 5 months with extremes ranging from 3 months to 3 years. The average age of onset was 9 months. Lesions were multiple yellowish papulo nodules of varying size and number. The diagnosis of xanthogranuloma was confirmed on biopsy with histological and immunohistochemical study in all our patients. Neurofibromatosis type 1 was found in only one patient, in whom the hemogram showed a major hyperleucocytosis with hypochromic microcytic anemia and osteo-medullary biopsy infiltration by non-langerhansian histiocytic cells. Ophthalmological examination done in 3 patients returned normal. Abdominal ultrasonography revealed hepatosplenomegaly in one patient and was normal in the other 3. Juvenile xanthogranuloma is a form of non-langerhansian histiocytosis. It most often affects young children. Clinically occurs in its classic form as papules or nodules yellow orange firm and well limited. The anatomopathological examination confirms the diagnosis. Interrogation and clinical examination should focus on finding extra skin lesions and signs of Neurofibromatosis type1. Ophthalmological examination is justified in case of ocular signs, in case of extra-cutaneous lesions or multiple lesions in a child under 2 years old. If combined with NF1, a blood count should be made to search for associated myelomonocytic leukemia.