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Smooth muscle hamartoma in Adult
Other Resources UpToDate PubMed

Smooth muscle hamartoma in Adult

Contributors: Craig N. Burkhart MD, Belinda Tan MD, PhD, Dean Morrell MD
Other Resources UpToDate PubMed

Synopsis

Congenital smooth muscle hamartomas develop during fetal life and are characterized by localized proliferations of smooth muscle bundles in the dermis with overlying vellus hairs. Congenital smooth muscle hamartomas may be noticed at birth or during early infancy. There are no systemic symptoms. The estimated prevalence is 1 in 2600 births with a slight male predominance.

Smooth muscle hamartomas are benign and, thus, the prognosis is excellent. Pigmentation and hair tend to become less noticeable with age.

Codes

ICD10CM:
Q85.9 – Phakomatosis, unspecified

SNOMEDCT:
239145008 – Diffuse smooth muscle hamartoma

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Michelin tire baby syndrome – May involve extensive involvement of hamartomas, or intellectual disability, seizures, and other developmental anomalies.
  • Becker nevus
  • Congenital melanocytic nevus
  • Connective tissue nevi

Best Tests

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Management Pearls

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Therapy

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References

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Last Updated:10/01/2018
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Smooth muscle hamartoma in Adult
A medical illustration showing key findings of Smooth muscle hamartoma : Asymmetric trunk or extremity distribution, Buttocks, Smooth plaque, Thigh, Trunk, Upper arm
Clinical image of Smooth muscle hamartoma - imageId=2396079. Click to open in gallery.  caption: 'A segmental patch of terminal hair growth on the back.'
A segmental patch of terminal hair growth on the back.
Copyright © 2024 VisualDx®. All rights reserved.