Juvenile Hyaline Fibromatosis: A 10-year Follow-up

• Published in: Indian journal of dermatology Vol. 62; no. 2; pp. 210 - 212
• Main Authors: Baltacioglu, Esra, Guzeldemir, Esra, Sukuroglu, Erkan, Yildiz, Kadriye, Yuva, Pinar, Aydin, Güven, Karacal, Naci
• Format: Journal Article
• Language: English
• Published: India Medknow Publications and Media Pvt. Ltd 01-03-2017; Medknow Publications & Media Pvt. Ltd; Medknow Publications & Media Pvt Ltd; Wolters Kluwer Medknow Publications
• Subjects: Care and treatment; Case Report; Case studies; Diagnosis; Fibroma; Gingival hyperplasia; gingivectomy; juvenile hyaline fibromatosis; India; juvenile hyaline fibromatosis; Gingival hyperplasia; gingivectomy
• ISSN: 0019-5154; 1998-3611
• DOI: 10.4103/ijd.IJD_166_16

Juvenile hyaline fibromatosis (JHF) is a rare hereditary disease with an autosomal recessive transmission. JHF is characterized by papulonodular skin lesions, osteolytic bone lesions, flexural joint contractures, and gingival hyperplasia and usually diagnosed in infancy or early childhood. JHF is thought to be a disorder of collagen metabolism and characterized by homogenous amorphous eosinophilic material and fibrous tissue. We report the case of a 14-year-old male child with multiple papulonodular skin lesions, progressive flexion contractures of joints, and severe gingival hyperplasia, with a 10-year follow-up. Although the lesions were totally removed thrice during the last 10 years, they recurred rigorously.