title: Uncommon Presentation of a Common Histiocytic Tumor: A Rare Entity.
abstract: Juvenile xanthogranuloma (JXG) is the most common form of non-Langerhans cell histiocytic lesion, characterized by benign, usually asymptomatic, self-healing yellowish brown papulonodular lesions of skin and other organs in the absence of metabolic disorder. The cells of origin of JXG are dermal dendrocytic cells. Histopathologically there is dermal infiltration of foamy or non-foamy histiocytes with or without giant cell. Immunohistochemistry shows CD68 positivity with CD1a and S-100 negativity of lesional cells although S-100-positive JXG have been reported. JXG may be associated with neurofibromatosis type one (NF 1) with increased risk of juvenile chronic myelogenous leukemia and other hematological malignancies. Our case was S-100 immunoreactive multiple, cutaneous JXGs with NF 1 without any visceral involvement or malignant complication. We are presenting this case due to its rarity.
authors.affiliation: Department of Dermatology, Malda Medical College and Hospital, Malda, West Bengal, India.
authors.forename: Chinmay
authors.initials: C
authors.lastname: Kar
authors.affiliation: Department of Dermatology, Malda Medical College and Hospital, Malda, West Bengal, India.
authors.forename: Kapildev
authors.initials: K
authors.lastname: Das
authors.affiliation: Department of Dermatology, Malda Medical College and Hospital, Malda, West Bengal, India.
authors.forename: Jayanta K
authors.initials: JK
authors.lastname: Barua
keywords: Juvenile xanthogranuloma; S-100 protein; neurofibromatosis type one