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July 1990

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

Author Affiliations

From the Pediatric Dermatology Unit (Drs Zvulunov and Metzker), Departments of Pathology (Dr Rotem) and Neonatology (Dr Merlob), Beilinson Medical Center, Petah Tiqwa, Israel, and Tel Aviv (Israel) University Sackler School of Medicine.

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)