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The diagnostic quandary of hereditary haemorrhagic telangiectasia vs. CREST syndrome
Author(s) -
Lee J.B.,
BenAviv D.,
Covello S.P.
Publication year - 2001
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.2001.04462.x
Subject(s) - sclerodactyly , crest syndrome , telangiectasia , medicine , crest , dermatology , calcinosis , pathology , disease , calcification , connective tissue disease , autoimmune disease , physics , quantum mechanics
The distribution and clinical appearance of the telangiectasia in the CREST syndrome ( c alcinosis, R aynaud’s phenomenon, o e sophageal involvement, s clerodactyly, t elangiectasia) and hereditary haemorrhagic telangiectasia (HHT) are very similar. Several previously reported cases of the CREST syndrome simulating HHT illustrate this diagnostic quandary. We report a patient who met the diagnostic criteria for both the CREST syndrome and HHT, and discuss the distinguishing features of the two diseases, including the distinctive histopathological findings of telangiectasia in HHT.