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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.