Internal Medicine

0521779407-13 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:15

916 Localized Scleroderma

differential diagnosis

■Systemic scleroderma – associated with acral sclerosis, Raynaud’s

phenomenon, and internal organ involvement (pulmonary, renal)

■Acrodermatitis chronica atrophicans – seen in Europe, due to Bor-

relia burgdorferi transmitted by tick bite; begins on extremity

■Sclerodermatous graft-versus-host disease – manifestation of

chronic GVHD in patient with history of transplantation

management

What to Do First

■Assess stage of disease – early, active, late or “burned out”

■Assess degree of involvement

General Measures

■Physical therapy/education to maintain joint mobility

■Camouflage cosmetics

specific therapy

Indications

■Early or active disease

■Extensive involvement

■Involvement causing muscle atrophy or joint contractures

■Patient concerns, even if mild disease

Treatment Options

■High potency topical corticosteroids

➣Apply directly to involved areas twice daily; occlusion may be

attempted to increase effect

➣Recommended for active lesions; avoid in late, sclerotic lesions

➣More useful for limited involvement; if widespread involvement,

may be difficult to apply and may result in systemic absorption

of steroids

➣May rarely lead to atrophy of skin, striae, telangiectasias,

although uncommon if used only in involved areas

■Topical calcipotriene (Dovonex)

➣Use on active lesions with occlusion

➣May cause irritation

■Intralesional corticosteroids

➣Triamcinolone acetonide injected directly into lesions

➣Use only on active lesions

➣Use only for single or few localized lesions

➣Side effects include pain, and risk of more pronounced atrophy