Claw-hand deformity

In ML-III patients, stiffness of the hands and shoulders due to rheumatoid arthritis leads to claw-hand deformities in addition to short stature and scoliosis. 

By 6 months of age, psychomotor retardation is usually obvious. Joint immobility progresses with development of claw-hand deformities and kyphoscoliosis. Hepatomegaly is prominent, but splenomegaly is minimal. Corneal haziness may be present but is subtle, and corneal opacities due to the accumulation of storage material are not as striking as in Hurler syndrome. Examination of peripheral blood smears reveals the presence of abnormal inclusions in cells such as lymphocytes, and increased lysosomal enzyme activity in whole blood as well as cultured fibroblasts is confirmatory of I-cell disease. 

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