juvenile hyaline fibromatosis
Juvenile hyaline fibromatosis (JHF) is a rare soft tissue tumor, characterized by papulo-nodular skin lesions (especially around the head and neck), soft tissue masses, gingival hypertrophy, joint contractures, and osteolytic bone lesions in variable degrees. Joint contractures may cripple patients and delay normal motor development if occurring in infancy. Severe gingival hyperplasia can interfere with eating and delay dentition. Histopathology analysis of involved tissues reveals cords of spindle-shaped cells embedded in an amorphous, hyaline material. JHF is a mild form of infantile systemic hyalinosis.
MONDO:0002300
dermis tumor
is_a
MONDO:0006424
soft tissue neoplasm
is_a
MONDO:0009229
hyaline fibromatosis syndrome
is_a
MONDO:0019060
bone neoplasm
is_a
MONDO:0023603
hereditary disorder of connective tissue
is_a
MONDO:0100118
hereditary skin disorder
is_a
Molluscum fibrosum
exact
Murray-Puretic-Drescher syndrome
exact
Puretic syndrome
exact
mesenchymal dysplasia
exact