(Juvenile) Hyaline Fibromatosis
Clinical
- Autosomal recessive
- Presents in children usually under age 4 years
- Skin, subcutaneous, soft tissue masses
- Papulo-nodular lesions
- Most frequent sites: scalp, neck, hands
- Slow growing
- Gingival hypertrophy
- Painful flexion contractures
- Osteolytic defects on xray
- May have growth retardation
- May merge clinically and histologically with infantile systemic hyalinosis
- Local recurrences may occur but are not destructive
