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October 1980

Piebaldness With Hirschsprung's Disease

Author Affiliations

Tamil Nadu, India

Arch Dermatol. 1980;116(10):1102. doi:10.1001/archderm.1980.01640340012007

To the Editor.—  Piebaldness is a genetically determined disorder of pigmentation that may be associated with other neurological disorders. We observed a patient with piebaldness associated with Hirschsprung's disease. Such an association has not been reported earlier, to the best of our knowledge.

Report of a Case.—  The patient, a 4-month-old boy, was the fourth child in a nonconsanguineous marriage. Clinical and radiological evidence of Hirschsprung's disease was demonstrated at the age of 2 months. Family history indicated that the eldest brother of the patient had a white forelock, hypopigmented patches on the face, and Hirschsprung's disease, whichFig 1.—White forelock with depigmented patches on upper part of forearm and lower part of arm, with island of normal pigmentation.Fig. 2.—Spastic descending colon, sigmoid colon, and rectum, with proximally distended colon, are visible on barium enema examination. had been surgically corrected. The father of the child had a white forelock