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 CASE REPORT
Year : 2008  |  Volume : 74  |  Issue : 4  |  Page : 371--374

Juvenile hyaline fibromatosis and infantile systemic hyalinosis: Divergent expressions of the same genetic defect?


Department of Derrmatology, T.N. Medical College and B.Y.L. Nair Ch. Hospital, Mumbai Central, Mumbai - 400 008, India

Correspondence Address:
Mandeep Dhingra
Department of Dermatology, OPD-14, 'O' bldg. 2nd floor, T.N. Medical college and B.Y.L. Nair Ch.Hospital, Dr. A.L. Nair Road, Mumbai Central. Mumbai- 400 008
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0378-6323.42913

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We describe here a three year-old girl with classic clinical and histological features of juvenile hyaline fibromatosis. We found a history of similar skin findings in her eldest sister, in whom the disorder took a rapidly progressive and fatal course in the second year of life, suggesting either a very severe form of juvenile hyaline fibromatosis, or the possibility of infantile systemic hyalinosis. The similarities and differences between these two described types of hyalinoses have been reviewed in reference to the present report.






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Online since 15th March '04
Published by Wolters Kluwer - Medknow