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The CRST syndrome (calcinosis, Raynaud's phenomenon, sclerodactyly and telangiectasia)
The British Journal of Oral Surgery
|November 1, 1976
Insights
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) syndrome is defined and linked to systemic sclerosis. This review covers existing literature and presents a new case, highlighting diagnostic features of telangiectasia.
Area of Science:
- Vascular Medicine
- Genetics
- Dermatology
Background:
- Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CRST) syndrome is a rare genetic disorder.
- Its association with systemic sclerosis requires further elucidation.
- Telangiectasias are a notable clinical feature requiring differentiation from other conditions.
Abstract:
The CRST syndrome is defined, and its relation to systemic sclerosis described. The literature referring to the condition is reviewed and a further case reported. Clinical and diagnostic features of the telangiectasia are emphasised, particularly in comparison with those of the similar condition, hereditary haemorrhagic telangiectasia.