Preferred Label : Juvenile hyaline fibromatosis;
ICD-11 definition : Juvenile hyaline fibromatosis (JHF) is a genetic condition characterized by papulonodular
skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of
the large joints due to perivascular hyaline deposition. Typical diagnostic criteria
are multiple hyaline subcutaneous fibroma, filamentous tumors of the skin, gingival
fibromatosis, muscle contractures of the extremities and multiple osteolytic bone
destructions.;
Origin ID : 1890146024;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
Juvenile hyaline fibromatosis (JHF) is a genetic condition characterized by papulonodular
skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of
the large joints due to perivascular hyaline deposition. Typical diagnostic criteria
are multiple hyaline subcutaneous fibroma, filamentous tumors of the skin, gingival
fibromatosis, muscle contractures of the extremities and multiple osteolytic bone
destructions.
