Lipochondrodystrophy (Gargoylism, Hurler's Syndrome) with Specific Cutaneous Deposits

Abstract
Summary: A peculiar skin lesion in a 6 1/2 year old boy with gargoylism is described. Biopsy showed vacuolized cells just below the epithelial membrane. These cells had the same microscopic appearance as those appearing in the internal organs of patients with gargoylism who have died. They also reacted in the same manner to various stains. A considerably increased thickness of the dermis with abundant formation of hyaline and collagenous bundles was also observed.

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