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Congenital smooth muscle hamartoma (CSMH) is a rare cutaneous hamartomatous lesion most often located on the lumbosacral area or proximal limbs (but rarely on atypical areas such as scalp, eyelid or foot) and characterized by a disorganized proliferation of smooth muscle fibers of arrector pili presenting usually as a localized skin-colored or hyperpigmented plaque (up to 10 cm in diameter) with prominent vellus hairs (most common classic form) or less commonly by multiple skin-colored papules that can coalesce to form irregularly shaped plaques. With time, hyperpigmentation and vellus hairs usually diminish and neither malignant transformation nor associated systemic involvement has been reported.
Estimated prevalence: Unknown (Unknown prevalence).
No clinical trials have been registered for congenital smooth muscle hamartoma.
5 publications have been identified in PubMed for congenital smooth muscle hamartoma. Research spans Case Report / Case Series (100%).
Croix M (2026). [PMID: 41645685](https://pubmed.ncbi.nlm.nih.gov/41645685/). *Dermatol Reports*. [Case Report / Case Series]
Colmant C (2026). [PMID: 41669896](https://pubmed.ncbi.nlm.nih.gov/41669896/). *Pediatr Dermatol*. [Case Report / Case Series]
Meza V (2025). [PMID: 39692078](https://pubmed.ncbi.nlm.nih.gov/39692078/). *J Ultrasound Med*. [Case Report / Case Series]
Pal SS (2024). [PMID: 39391140](https://pubmed.ncbi.nlm.nih.gov/39391140/). *Int J Clin Pediatr Dent*. [Case Report / Case Series]
Data assembled from 3 of 12 sources · Last updated Sep 20, 2026, 5:50 AM UTC
European rare disease database
Genetic and Rare Diseases Info Center