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July 1972

Calcifying Epithelioma of MalherbeAssociation With Myotonic Muscular Dystrophy

Author Affiliations


From the Division of Medical Genetics, Johns Hopkins University School of Medicine, Baltimore. Dr. Harper is now with the University Hospital of Wales, Cardiff.

Arch Dermatol. 1972;106(1):41-44. doi:10.1001/archderm.1972.01620100029008

During a genetic study of myotonic dystrophy, seven individuals were found with histologically proven benign calcifying epitheliomas of Malherbe. In one family, mother and son were affected with both conditions; in another, two siblings were affected. Four patients had multiple tumors. No tumors were found in relatives without muscle disease. It is likely that the occurrence of calcifying epithelioma represents a further pleiotropic effect of the myotonic dystrophy gene; since this tumor is frequently mistaken clinically for a simple sebaceous cyst, its occurrence in myotonic dystrophy may be more common than is apparent.