Approximately 40 cases of juvenile hyaline fibromatosis have been reported to date. This condition has not previously been described in the plastic surgery literature. This report describes a case where the disease led to a complete nasal obstruction due to progressive involvement of the nose, necessitating surgical intervention. The clinical and pathological aspects of the disease, and the differential diagnosis (ie, congenital generalized fibromatosis, Von Recklinghausen disease, Winchester syndrome) are discussed.
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MoyLS, MoyRL, MatsuokaLY, OhtaA, UittoJ. Lipoid proteinosis: ultrastructural and biochemical studies. J Am Acad Dermatol1987; 16: 1193–201.
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BedfordCD, SillsJA, Sommelet-OliveD, BomanF, BeltramoF, CornuG. Juvenile hyaline fibromatosis: a report of two severe cases. J Pediatr1991; 119: 404–10.
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IwataS, HoriuchiR, MaedaH, IshikawaH. Systemic hyalinosis or juvenile hyaline fibromatosis. Ultrastructural and biochemical study of cultured skin fibroblasts. Arch Dermatol Res1980; 267: 115–21.
