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July 1990

Congenital Smooth Muscle Hamartoma: Prevalence, Clinical Findings, and Follow-up in 15 Patients

Am J Dis Child. 1990;144(7):782-784. doi:10.1001/archpedi.1990.02150310050024

• Congenital smooth muscle hamartoma is a congenital skin lesion characterized by proliferation of bundles of smooth muscle within the reticular dermis. We report on a group of 15 children with this lesion, confirmed by skin biopsy specimen, its prevalence, the occurrence of associated anomalies, and long- term follow-up. To our knowledge, this is the largest group of patients with this lesion described in the literature. The estimated prevalence is about 1:2600 live births with slight male predominance. The lesion is most frequent in the lumbosacral area (67% [10/15]) and a positive pseudo-Darier's sign is present in 80% (12/15) of patients. During the follow-up period of up to 7 years, the lesions enlarged slightly but became less prominent. No malignant transformation was observed.

(AJDC. 1990;144:782-784)