منابع مشابه
Hyaline fibromatosis syndrome: cutaneous manifestations.
Hyaline fibromatosis syndrome is the current name for clinical manifestations of diseases previously known as "infantile systemic hyalinosis" and "juvenile hyaline fibromatosis". The authors report representative clinical cases of each one of the above subtypes with emphasis on cutaneous manifestations and difficulties for early diagnosis in this syndrome, essentially of multidisciplinary appro...
متن کاملHyaline Fibromatosis Syndrome: A Rare Inherited Disorder
Hyaline fibromatosis syndrome (HFS) is rare autosomal recessive disease characterized by the deposition of amorphous hyaline material in skin and visceral organs. It represents a disease spectrum with infantile systemic hyalinosis (ISH) being the severe form and juvenile hyaline fibromatosis (JHF) being the mild form. Dermatologic manifestations include thickened skin, perianal nodules, and fac...
متن کاملJuvenile Hyaline Fibromatosis
Juvenile hyaline fibromatosis (JHF) is a rare, progressive autosomal recessive disease that's characterized by papulonodular skin lesions, soft tissue masses, joint contractures, gingival hypertrophy and osteolytic bone lesions. We present here the case of a 2-yr-old boy with JHF along with a review of the relevant literature. This case demonstrates that JHF should be considered in the differen...
متن کاملJuvenile hyaline fibromatosis.
Juvenile byaline fibromatosis is a rare connective tissue disorder, characterized by skin tumours, gingival hypertrophy and flexion contractures of the acral joints. Other associations include stunted growth, osteolytic changes and muscle weakness. The mental development is normal. Juvenile hyaline fibromatosis probably has an autosomal recessive inheritance, characterized by increased synthesi...
متن کاملJuvenile hyaline fibromatosis.
J Postgrad Med 2004;50:125-6 n 18-month-old female child born of a second degree consanguineous marriage presented with asymptomatic, pink, raised lesions on the body since the age of six months and boggy swellings on the scalp since the age of 12 months. There was a gradual increase in the size of the skin and scalp lesions. A progressive, painless, flexion deformity of both the upper and lowe...
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ژورنال
عنوان ژورنال: Indian Journal of Pediatrics
سال: 2021
ISSN: ['0019-5456', '0973-7693']
DOI: https://doi.org/10.1007/s12098-021-03724-6