Document Detail


Progressive nodular histiocytoma associated with thrombocytopenia with absent radii (TAR syndrome) and angiofibromas.
MedLine Citation:
PMID:  16874423     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A 50-year-old woman was admitted due to a long-standing history of cutaneous lesions, which were gradually increasing in number and size, located on the trunk and extremities. Histological studies confirmed the initial clinical diagnosis of histiocytomas. Moreover, the patient had numerous smooth erythematous papules on her chin and around her nose, which were diagnosed histologically as angiofibromas. The patient had congenital phocomelia. Analytical and imaging studies revealed the presence of bilateral phocomelia due to absent radii and thrombocytopenia (TAR syndrome). Multiple histiocytomas in a normolipaemic patient bring up several differential diagnoses. Slow progressive evolution without spontaneous resolution and a scattered distribution on the trunk and extremities suggest the diagnosis of progressive nodular histiocytoma. To our knowledge progressive nodular histiocytoma has not been reported previously associated either with TAR syndrome or with angiofibromas. These entities are uncommon, thus their association may not be due to chance.
Authors:
Elena Rosón; Angeles Flórez; Carlos Feal; Carlos De La Torre; Ignacio García-Doval; Teresa Abalde; Manuel Cruces
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Acta dermato-venereologica     Volume:  86     ISSN:  0001-5555     ISO Abbreviation:  Acta Derm. Venereol.     Publication Date:  2006  
Date Detail:
Created Date:  2006-07-28     Completed Date:  2006-11-09     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370310     Medline TA:  Acta Derm Venereol     Country:  Norway    
Other Details:
Languages:  eng     Pagination:  348-50     Citation Subset:  IM    
Affiliation:
Department of Dermatology, Hospital Provincial, Complejo Hospitalario de Pontevedra, Spain. eroson64@yahoo.es
Data Bank Information
Bank Name/Acc. No.:
OMIM/274000
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MeSH Terms
Descriptor/Qualifier:
Angiofibroma / complications*,  pathology
Ectromelia / complications*
Female
Histiocytoma / complications,  pathology*
Humans
Middle Aged
Radius / abnormalities*
Skin Diseases / complications,  pathology*
Skin Neoplasms / complications,  pathology
Syndrome
Thrombocytopenia / complications*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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