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Table 1 Variants of localized scleroderma recently classified by Tuffanelli [2]

From: Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications

I.   Localized scleroderma

   a) Morphea [plaque-type; guttate; profound; nodular; bullous; Atrophoderma of Pasini and Pierini]

   b) Linear scleroderma ["en coup de sabre"; with facial atrophy (Parry-Romberg syndrome); acral; with arthritis, myositis, and growth defects of bone; melorheostotic]

   c) Generalized morphea [pansclerotic; edematous; with lichen sclerosus]

II.   Localized scleroderma as a component of an overlap syndrome

   a) Mixed connective tissue disease

   b) Undifferentiated connective tissue disease

   c) Sclerodermatomyositis

III.   Localized scleroderma as a component of eosinophilic fasciitis (Shulman's syndrome)