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Juvenile Hyaline Fibromatosis: A 10-year Follow-up

Esra Baltacioglu1, Esra Guzeldemir2, Erkan Sukuroglu1

  • 1Department of Periodontology, Faculty of Dentistry, Karadeniz Technical University, Trabzon, Turkey.

Indian Journal of Dermatology
|April 13, 2017
PubMed

Insights

Juvenile hyaline fibromatosis (JHF) is a rare genetic disorder causing skin lesions and joint contractures. This case highlights the persistent recurrence of JHF symptoms despite multiple surgical interventions over a decade.

Area of Science:

  • Genetics
  • Dermatology
  • Pathology

Background:

  • Juvenile hyaline fibromatosis (JHF) is a rare, autosomal recessive hereditary condition.
  • It typically manifests in infancy or early childhood with characteristic papulonodular skin lesions, bone abnormalities, joint contractures, and gingival hyperplasia.
  • Pathologically, JHF is associated with abnormal collagen metabolism, presenting as amorphous eosinophilic material and fibrous tissue.

Purpose of the Study:

  • To report a unique case of Juvenile hyaline fibromatosis (JHF) in a 14-year-old male.
  • To document the long-term clinical course and management challenges of JHF.
  • To emphasize the aggressive recurrence pattern of JHF lesions.

Main Methods:

  • Case report of a 14-year-old male diagnosed with Juvenile hyaline fibromatosis (JHF).
  • Clinical observation over a 10-year follow-up period.
  • Documentation of surgical interventions and lesion recurrence.

Main Results:

  • The patient presented with multiple papulonodular skin lesions, progressive joint flexion contractures, and severe gingival hyperplasia.
  • Despite three instances of complete lesion removal over 10 years, rigorous recurrence was observed.
  • The case underscores the challenging and persistent nature of JHF.

Conclusions:

  • Juvenile hyaline fibromatosis (JHF) presents significant challenges due to its relentless recurrence.
  • Long-term follow-up is crucial for managing JHF patients.
  • Further research into the underlying collagen metabolism defects in JHF is warranted.

Keywords:
Gingival hyperplasiagingivectomyjuvenile hyaline fibromatosis

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