Lipoid Proteinosis

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Abstract
Lipoid proteinosis is a rare form of storage disease characterized by the deposition of an abnormal hyaline-like material primarily in the skin and mucous membranes. Approximately 100 cases have been recorded in the literature.2At least one-third have been distributed across Europe and America. The following case emphasizes the principal clinical and histologic features of this disease. Report of a Case A 2½-year-old white girl was admitted to the Children's Service of the Massachusetts General Hospital for evaluation of dysphonia, alopecia, and unusual skin lesions. The patient's mother noticed that the child had a high-pitched hoarse cry since early infancy. A barium swallow performed at an outside hospital at 1 year of age was interpreted as normal. Four months prior to admission, erythematous, pruritic, maculopapular cutaneous lesions appeared on the central region of the face. Within 2 months, welldemarcated, slightly infiltrated brown lesions with central ulceration developed on the

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