The diagnostic quandary of hereditary haemorrhagic telangiectasia vs. CREST syndrome.
The distribution and clinical appearance of the telangiectasia in the CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal involvement, sclerodactyly, telangiectasia) 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.
Lee
J B
JB
Ameripath Laboratory, 10th Floor, 145 East 32nd Street, New York, NY 10016, USA. jboklee@netscape.net
Ben-Aviv
D
D
Covello
S P
SP
D017675:CREST Syndrome / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008875:Middle Aged; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000175:diagnosis* / Q000473:pathology