Volume 24, Number 5 (Monthly August 2013)                   J Urmia Univ Med Sci 2013, 24(5): 373-378 | Back to browse issues page


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Amirnia M, Zeinali F. A CHILD WITH JUVENILE HYALINE FIBROMATOSIS: A CASE REPORT . J Urmia Univ Med Sci. 2013; 24 (5) :373-378
URL: http://umj.umsu.ac.ir/article-1-1775-en.html

Resident of Dermatology, Tabriz University of Medical Sciences, Sina Hospital, Tabriz, Iran , drs_zeinali@yahoo.com
Abstract:   (4850 Views)
 

Received: 1 Apr, 2013 Accepted: 13 Jun, 2013

 

Abstract

 

Juvenile hyaline fibromatosis is a rare genetic disease that is characterized by multiple papulonudular skin lesions, gingival hypertrophy and joint contracture. According to our knowledge, less than 70 cases with features suggesting the diagnosis have been reported. It is an autosomal recessive disease that is usually detected in infancy or early childhood.

 

We report a 14-year old boy, who presented skin colored to erythematous painless rubbery nodules on the scalp, face, hand, and feet and flexion contracture on both elbow and gingival hypertrophy.

 

Radiological findings showed erosion at the end of distal phalanxes with marginal sclerosis and soft tissue masses on toes. Pathological finding were an increased number of fibroblasts embedded in an amorphous eosinophilic hyalinized matrix. Some stoma cells were observed in the lacuna-like spaces (chondroid cell) within this material.

 

For our patient, the diagnosis of JHF was based on the presence of typical tumoral lesions, gingival hyperplasia, joint contracture, and the characteristic histopathological findings. The presence of amorphous hyaline material in the dermis supported our diagnosis of JHF.

 

 
Full-Text [PDF 383 kb]   (949 Downloads)    
Type of Study: case report | Subject: آناتومی
Received: 2013/08/6 | Accepted: 2013/08/12 | Published: 2013/08/12

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