CONGENITAL SMOOTH MUSCLE HAMARTOMA

By Gary M. White, MD


Congenital smooth muscle hamartoma (CSMH) is a benign cutaneous lesion caused by an overabundance of mature smooth muscle fibers in the dermis.

Clinical

A slightly pigmented, hypertrichotic area on the trunk or extremities is characteristic. Stroking of the lesions causes contraction of the smooth muscle bundles resulting in induration or piloerection, the so called "pseudo Darier's sign". The lumbosacral location is the preferred site. Rarely, other clinical appearances may be seen, e.g. a patch with perifollicular papules, vermiform movements, a congenital linear, reddish, atrophic plaque [PD 1996;13;222], or Michelin tire baby.

Treatment

No treatment is indicated as lightening of the color and reduction of the hypertrichosis usually occur over time.

References

Congenital, hypertrichotic plaques. Indian Journal of Dermatology Jan-Mar 2009

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