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Right arrow Allergy & Dermatology

PEDIATRICS Vol. 104 No. 1 July 1999, pp. 113-115

EXPERIENCE AND REASON:
A Case of Congenital Multiple Myofibromatosis Developing in an Infant

Received Dec 14, 1998; accepted Feb 11, 1999.

Barbara J. Schrodt and Jeffrey P. Callen

University of Louisville Department of Medicine Division of Dermatology Louisville, KY 40202

Background.  Infantile myofibromatosis is marked by the development of firm, discrete, flesh-colored to purple nodules in skin, muscle, bone, and/or subcutaneous tissues. In cases without visceral involvement, the prognosis is excellent with expected spontaneous regression of nodules in 1 to 2 years. Visceral lesions are associated with significant morbidity and mortality generally within the first few months of life secondary to obstruction of a vital organ, failure to thrive, or infection.

Observation.  We present a case of congenital myofibromatosis initially presenting as a single, asymptomatic nodule on the midback of an otherwise healthy 1-month-old white boy. Over the subsequent 6 months, the child developed a left-sided head tilt with the formation of additional myofibromas of the skin and musculature, but without visceral involvement. Physical examinations have continued to show age-appropriate growth and development.

Conclusion.  Clinicians should be aware of this rare but potentially life-threatening entity and consider infantile myofibromatosis in the differential diagnosis of pediatric dermal and subcutaneous nodules, particularly when associated with a new onset of head tilt. Close clinical follow-up is recommended in all cases of infantile myofibromatosis.  Key words:  infantile myofibromatosis, leiomyoma, head tilt.




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O. G. Dimson, B. A. Drolet, J. F. Southern, A. Rock, A. L. Winthrop, and N. B. Esterly
Congenital Generalized Myofibromatosis in a Neonate
Arch Dermatol, May 1, 2000; 136(5): 597 - 600.
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