Document Detail

Primary and secondary intralymphatic histiocytosis.
MedLine Citation:
PMID:  24440319     Owner:  NLM     Status:  Publisher    
BACKGROUND: Intralymphatic histiocytosis (IH) is a rare condition often associated with systemic disease. A benign condition, clinical presentations can vary greatly and its cause is largely unknown. Histologically, there are macrophages within distended lymphatic vessels, although this can be an incidental finding or the primary abnormality.
OBJECTIVE: We present a series of 7 cases of IH with and without disease associations, and a review of the literature. We propose IH as either primary (without associated conditions) or secondary (associated with systemic disease).
METHODS: This was a retrospective collection of patients whose skin biopsy specimens revealed intralymphatic collections of histiocytes. We reviewed their clinical presentation, disease associations, and staining of slides with CD68 in all cases, D2-40 in 5 cases, and HLA-DR in 4 cases.
RESULTS: Clinical features were highly variable, and not all cases were associated with systemic disease. One case had admixed reactive angioendotheliomatosis. All 4 cases stained for HLA-DR showed strong expression by the intralymphatic macrophages.
LIMITATIONS: Retrospective analysis and limited numbers are limitations.
CONCLUSION: IH is not always associated with systemic disease although macrophage activation nevertheless implies immune activation.
Farrah Bakr; Naomi Webber; Hiva Fassihi; Victoria Swale; Fiona Lewis; Ed Rytina; Galia Tamar Ben-Zvi; Paul Norris; Olivia Espinosa; Sunanda Dhar; Paul Craig; Alistair Robson
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-1-16
Journal Detail:
Title:  Journal of the American Academy of Dermatology     Volume:  -     ISSN:  1097-6787     ISO Abbreviation:  J. Am. Acad. Dermatol.     Publication Date:  2014 Jan 
Date Detail:
Created Date:  2014-1-20     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7907132     Medline TA:  J Am Acad Dermatol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.
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