Clinical manifestation

Onset of symptoms from age 2-4 years; signs and symptoms similar to those of Hurler syndrome, but milder, with slower progression; lichenified, dry, thick skin with diminished elasticity; increased pigmentation on the dorsum of the hands; sclerodermalike changes; hypertrichosis of the extremities; pale colored hair; mild skeletal deformation and deformity of the hands; growth sometimes normal; aortic stenosis or regurgitation sometimes present; hepatosplenomegaly; intelligence usually normal

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