Related Experiment Video
Updated: Aug 12, 2026
08:22
Assessment and Characterization of Hyaloid Vessels in Mice
Published on: May 15, 2019
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
Summary
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.
Related Concept Videos
01:20The Retinoblastoma Gene
Tumor suppressor genes are normal genes that can slow down cell division, repair DNA mistakes, or program the cells for apoptosis in case of irreparable damage. Hence, they play an essential role in preventing the proliferation of damaged cells.
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
01:27Growth of Cartilage and Bone Tissue
Chondrocytes form a temporary cartilaginous model by dividing and secreting a thick gel-like extracellular matrix. Once the chondrocytes undergo programmed cell death, osteoblasts enter the site of the cartilaginous model. The process of replacing the temporary cartilaginous model with bone in an ordered manner is called endochondral ossification. In endochondral ossification, not all of the cartilage is replaced by bone tissue. Some cartilage that performs a protective and supportive function...
01:20The Retinoblastoma Gene
Tumor suppressor genes are normal genes that can slow down cell division, repair DNA mistakes, or program the cells for apoptosis in case of irreparable damage. Hence, they play an essential role in preventing the proliferation of damaged cells.
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
01:31Bone Remodeling and Repair
Osteoclasts are cells responsible for bone resorption and remodeling. They originate from hematopoietic progenitor cells present in the bone marrow. Numerous progenitor cells fuse to form multinucleated cells, each with 10-20 nuclei. A single osteoclast has a diameter of 150 to 200 µM. These cells have ruffled borders that break down the underlying bone tissue and release minerals such as calcium into the blood in bone resorption. Osteoclasts cling to bones with their ruffled edges during bone...