Eponymous Syndromes

Question 1

What is CREST

Answer 1

Multi system autoimmune connective tissue disorder
Part of scleroderma family
Features incl:

CALCINOSIS

• pathological calcification of soft tissues
• tender white chalky deposits, may ulcerate
• typically on flexor surfaces hands / extensor surfaces forearm & knees
• may be subclinical

RAYNAUDS

• described by Raynaud in 1862
• episodic triphasic colour changes (pallor>cyanosis>rubor)
• typically affecting hands in response to cold/emotional stress
• assoc with normal proximal pulses

ESOPHAGEAL DYSMOTILITY
- typically dysphagia and reflux

SCLERODACTYLY

• thickening of digits
• tapered appearance distal > proximal

TELANGECTASIA
- may be multiple esp face, upper trunk, hands (+ mucosal)