at least one extracutaneous manifestation In addition, though rare, progression to juvenile systemic sclerosis (JSSc) has been reported TABLE 101.4 CLASSIFICATION OF SYSTEMIC SCLEROSIS, LOCALIZED SCLERODERMAS, AND SCLERODERMA-LIKE DISORDERS Systemic sclerosis Cutaneous scleroderma Diffuse Limited Overlap syndromes Sclerodermatomyositis or with other connective tissue diseases Mixed connective tissue disease (MCTD) Localized scleroderma Circumscribed morphea Generalized morphea Pansclerotic morphea Linear (include “en coup de sabre”) morphea Mixed subtype Graft-versus-host disease Chemically induced scleroderma-like disease Polyvinyl chloride Toxic oil syndrome Pentazocine Bleomycin Adjuvant disease Pseudosclerodermas Phenylketonuria Syndromes of premature aging Localized idiopathic fibroses Scleredema Diabetic cheiroarthropathy Porphyria cutanea tarda Reprinted from Zulian F, Cassidy JT The systemic sclerodermas and related disorders In: Cassidy JT, Petty RE, Laxer RM, et al., eds Textbook of Pediatric Rheumatology 6th ed Philadelphia, PA: Saunders Elsevier; 2011:414– 437 Copyright © 2011 Elsevier With permission