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Infantile myofibromatosis - Skin
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Infantile myofibromatosis - Skin

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Contributors: Craig N. Burkhart MD, Dean Morrell MD, Lowell A. Goldsmith MD, MPH, Nancy Esterly MD
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Synopsis

Infantile myofibromatosis is a rare mesenchymal disorder in which benign fibroblasts proliferate and invade adjacent structures. A majority of cases present within the first 2 years of life (90%), and 60% present at birth. There are two forms of the disease, solitary and multicentric; the latter can have visceral involvement. The solitary form accounts for a majority of cases (70%). The multicentric form appears more often at birth and in females.

In the solitary form lesions are seen in the skin, subcutaneous tissue, or muscle. In the multicentric form lesions can be seen in the bone and viscera. Skeletal lesions occur in more than half the cases, with the skull, femur, tibia, spine and ribs involved most commonly.

Without visceral involvement the prognosis is favorable: it is typically benign and self-limited and has a low recurrence rate. The multicentric form with visceral involvement is less favorable, with a 73% fatality rate, typically due to cardio-pulmonary failure.

Familial cases are rare, but autosomal-dominant and recessive inheritance have been reported.

Most of these lesions in the past were called hemangiopericytomas.

For more information on infantile myofibromatosis 1, see OMIM.

For more information on infantile myofibromatosis 2, see OMIM.

Codes

ICD10CM:
Q89.8 – Other specified congenital malformations

SNOMEDCT:
254146000 – Infantile myofibromatosis

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Last Updated: 03/29/2017
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Infantile myofibromatosis - Skin
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Infantile myofibromatosis (Solitary Type) : Head/neck, Skin ulcer, Trunk, Solitary lesion
Clinical image of Infantile myofibromatosis
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