[Skip to Navigation]
January 1958

Gargoylism: A Review Including Two Occurrences in the American Negro

Author Affiliations

Washington, D.C.
From the Department of Pathology, Children's Hospital; formerly Fellow in Pathology (Dr. Gilbert, present address, Austin, Texas) and Associate Pathologist (Dr. Guin), Children's Hospital, Washington, D. C.

AMA Am J Dis Child. 1958;95(1):69-80. doi:10.1001/archpedi.1958.02060050071011

Gargoylism was first reported by Hunter1 in 1917, but it was not until two years later that the disease was described in greater detail by Hurler.2 Clinically, the disease is characterized by skeletal dystrophy, coarse facial features, mental retardation, enlargement of the liver and spleen, and clouding of the corneas. When all of the stigmata are present, the disease is readily recognized. However, those cases lacking some of the characteristic criteria are classified as formes frustes.

The purpose of this paper is to present five cases, two of which were found in Negro children. The latter are believed to be the first documented examples of gargoylism in the American Negro. In addition, a review of the literature has been made, as well as a summary of the recent advances in the recognition and understanding of the fundamental defects of this disease process.

Report of Cases  Case 1.—A 4-year-old

Add or change institution