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August 1989

Picture of the Month

Author Affiliations

Contributed from the Department of Pediatrics, Medical University of South Carolina, Charleston.

Am J Dis Child. 1989;143(8):963-964. doi:10.1001/archpedi.1989.02150200123031

Denouement and Discussion  Congenital rhabdomyomatous mesenchymal hamartoma of the skin, to our knowledge, has been described once previously as a single pedunculated appendage on the chin of a new-born. In that case, the appendage had an abnormal arrangement of dermal mesenchymal elements, with a prominence of skeletal muscle. In the patient shown herein, the connective-tissue core of the pulp contained adipose cells and striated muscle bundles that were oriented primarily parallel to the long axis. The epidermis was slightly hyperplastic and under higher magnification demonstrated striated muscle bundles. Pathologically, it was characterized as a hamartoma because of the abnormal mixture of normal cells (ie, striated muscle, mesenchyme, and skin) in an appropriate area, the face muscles of expression.Other than a very unusual appearance, the striking feature was the random contractile movements of the appendages, giving a snakelike appearance. Computed tomographic scan revealed no involvement of

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