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Stiff skin syndrome (SSS) is a rare disorder characterized by progressive rock-hard induration of the skin. In this review, the authors describe 6 previously unreported cases and summarize findings in these patients and in an additional 31 from the literature.
They describe a typical presentation in infancy or early childhood of insidious skin hardening with well-demarcated, rock-hard skin bound firmly to the underlying tissues. The buttocks, thighs, pelvic region, and shoulder girdle are the most commonly affected areas. The overlying skin is normal or has mild hypertrichosis and hyperpigmentation. Extracutaneous features may develop, including joint stiffness and contractures (especially in large joints), scoliosis, a tiptoe gait or limp, …