0521779407-19 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:21
1328 SclerodermaSCLERODERMA
GEORGE MOXLEY, MD
history & physical
History
■Systemic sclerosis in two forms
➣Diffuse scleroderma
➣CREST syndrome (calcinosis, Raynaud’s, esophageal dysmotility,
sclerodactyly, telangiectasia)
■Possible association w/ exposure to silica dusts, organic solvents
(vinyl chloride). Epidemiologic evidence shows no association w/
silicone breast implants.
■Localized scleroderma (morphea, linear scleroderma) – involves
only skin, subcutaneous tissue & muscle. No Raynaud’s or systemic
disease.Signs & Symptoms
■Raynaud’s phenomenon (finger pain & numbness upon exposure
to cold or stress, w/ two or three shades of discoloration [red,
white, purplish]) is hallmark & usually first symptom. Raynaud’s w/o
underlying disease also extremely common. Associated w/ expo-
sure to bleomycin, ergot alkaloids, beta blockers, methysergide, vinyl
chloride, vibrating tools. Sometimes leads to digital ulcers or gan-
grene. 13% of pts w/ Raynaud’s eventually develop an inflammatory
rheumatic disease.
■Skin hypopigmentation over clavicular areas, hyperpigmentation
over distal arms
■Subcutaneous hard nodules around joint capsules (calcinosis, sub-
cutaneous calcific deposits)
■Reflux esophagitis (due to esophageal dilatation & hypomotility)
■Contracted tight thickened skin–sclerodactyly in fingers, also fore-
arms, face, trunk. Pursed mouth, reduced oral aperture so gap be-
tween upper & lower teeth <2 cm.
■Telangiectasias on lips, face, hands
■Joint pain & stiffness (mild inflammatory arthropathy). Sometimes
friction rubs of tendons within tendon sheaths.
■Resorption of terminal phalanges & overlying digital soft tissue
■Fatigue, simple myopathy or inflammatory myositis w/ weakness
■Dry mouth & dry eyes (Sjogren’s syndrome)