Two siblings with juvenile hyaline fibromatosis: case reports and review of the literature

In this paper, we describe two siblings with Juvenile Hyaline Fibromatosis (JHF) who were diagnosed at the age of 34 and 29 years respectively. JHF is a very congenital disease, mainly diagnosed in the first few years of life, with less than 40 published cases in literature. All the main clinical fe...

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Bibliographic Details
Published inClinical rheumatology Vol. 18; no. 3; pp. 248 - 252
Main Authors Keser, G, Karabulut, B, Oksel, F, Calli, C, Ustün, E E, Akalin, T, Koçanaoğullari, H, Gümüdiş, G, Doğanavşargil, E
Format Journal Article
LanguageEnglish
Published Germany Springer Nature B.V 01.01.1999
Subjects
Adult
Family Health
Female
Fibroma - congenital
Fibroma - diagnostic imaging
Fibroma - pathology
Fibromatosis, Gingival - pathology
Humans
Male
Nuclear Family
Radiography
Soft Tissue Neoplasms - congenital
Soft Tissue Neoplasms - diagnostic imaging
Soft Tissue Neoplasms - pathology
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Summary:In this paper, we describe two siblings with Juvenile Hyaline Fibromatosis (JHF) who were diagnosed at the age of 34 and 29 years respectively. JHF is a very congenital disease, mainly diagnosed in the first few years of life, with less than 40 published cases in literature. All the main clinical features of this syndrome, which may be summarised as multiple subcutaneous tumours, marked gingival hypertrophy, flexion contractures and osteolytic lesions were present in both of these cases. Clinical, radiological and histological differential diagnosis of JHF were made. Recent information about histopathology, treatment and prognosis of JHF was also reviewed.
Bibliography:ObjectType-Case Study-3
SourceType-Scholarly Journals-1
content type line 23
ObjectType-Review-1
ObjectType-Feature-5
ObjectType-Report-2
ObjectType-Article-4
ISSN:0770-3198
1434-9949
DOI:10.1007/s100670050094