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Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
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CASE REPORT
Year : 2017  |  Volume : 62  |  Issue : 2  |  Page : 210-212

Juvenile hyaline fibromatosis: A 10-year follow-up


1 Department of Periodontology, Faculty of Dentistry, Karadeniz Technical University, Trabzon, Turkey
2 Department of Periodontology, Faculty of Dentistry, Kocaeli University, Kocaeli, Turkey
3 Department of Pathology, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey
4 Beytepe Hospital Dental Clinic, Hacettepe University, Ankara, Turkey
5 Department of Plastic and Reconstructive Surgery, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey

Correspondence Address:
Esra Baltacioglu
Department of Periodontology, Faculty of Dentistry, Karadeniz Technical University, Kanuni Kampusu, Trabzon 61080
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_166_16

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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.


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