Langerhans Cell Histiocytosis Evolving into Juvenile Xanthogranuloma: Two Linked Entities. (April 2023)

• Record Type: Journal Article
• Authors: Shaker, Nada; Sangueza, Omar P.
• Is Part Of: American journal of dermatopathology. Volume 45:Number 4(2023)
• Page Start: 259
• Page End: 263
• Publication Date: 2023-04
• Subjects: Langerhans cell histiocytosis -- juvenile xanthogranuloma; Skin -- Diseases -- Periodicals; Histology, Pathological -- Periodicals; 616.50705
• DOI: 10.1097/DAD.0000000000002394
• Languages: English
• ISSNs: 0193-1091

Abstract

Abstract : Background: Langerhans cell histiocytosis (LCH) represents a myeloid clonal proliferation that involves the skin and other organs. Occasionally, cases of LCH evolve into juvenile xanthogranuloma (JXG). Case Presentation: A 7-month-old boy presented with an itchy, flaky rash resembling seborrheic dermatitis affecting the scalp and eyebrows. The lesions started at 2 months old. On physical examination, there were reddish/brown lesions on the trunk, denuded areas on the groin and neck, and a large lesion behind his bottom teeth. In addition, there were thick white plaques in his mouth and thick whitish material in both ears. A skin biopsy showed features of LCH. Radiologic examination demonstrated several osteolytic lesions. Chemotherapy produced marked improvement. A few months later, the patient developed lesions with clinical and histologic features of XG. Conclusion: A possible association between LCH and XG is explained by lineage maturation development. Chemotherapy may play a role in modifying the production of cytokines that enhance the transformation or 'maturation' of Langerhans cells into multinucleated macrophages (Touton cells) characteristic of a more favorable proliferative inflammatory condition.