Document Detail

Langerhans cell histiocytosis and juvenile xanthogranuloma. Two case reports.
MedLine Citation:
PMID:  15237270     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Histiocytoses represent a large, puzzling group of diseases which may involve the skin and other organs. At present, juvenile xanthogranuloma is the disorder most often confused with Langerhans cell histiocytosis. A complex overlap exists between juvenile xanthogranuloma and Langerhans cell histiocytosis, with lesions showing clinical and/or pathological features of both disorders. OBSERVATIONS: We report 2 patients affected by Langerhans cell histiocytosis who, during chemotherapy, presented cutaneous lesions with clinical and histological features of juvenile xanthogranuloma. During the therapy, in both cases, histological examination of new biopsies revealed the presence of Touton giant cells in the dermis with a few histiocytic cells; immunohistochemical staining was negative for CD1a, and no Birbeck granules were seen by ultrastructural examination. RESULTS AND CONCLUSION: A possible explanation for the link between Langerhans cell histiocytosis and juvenile xanthogranuloma regards the lineage development and the relationships of histiocytes. We suggest that chemotherapy can modify the production of cytokines by influencing the conversion or 'maturation' of pathological cells into macrophages or xanthomatous cells and fusing them to form multinucleated giant Touton cells. In our opinion, the modification of the cutaneous lesions during chemotherapy in Langerhans cell histiocytosis patients, as observed in our cases, could be a favorable prognostic factor.
Annalisa Patrizi; Iria Neri; Federica Bianchi; Valentina Guerrini; Cosimo Misciali; Giuseppina Paone; Roberta Burnelli
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Dermatology (Basel, Switzerland)     Volume:  209     ISSN:  1018-8665     ISO Abbreviation:  Dermatology (Basel)     Publication Date:  2004  
Date Detail:
Created Date:  2004-07-06     Completed Date:  2004-10-07     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9203244     Medline TA:  Dermatology     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  57-61     Citation Subset:  IM    
Copyright Information:
Copyright 2004 S. Karger AG, Basel
Department of Clinical and Experimental Medicine, Division of Dermatology, University of Study of Bologna, Bologna, Italy.
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MeSH Terms
Diagnosis, Differential
Histiocytosis, Langerhans-Cell / complications,  diagnosis*,  pathology
Xanthogranuloma, Juvenile / complications,  diagnosis*,  pathology

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