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Accession number;06A0338789
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| Title;A Case of Juvenile Hyaline Fibromatosis |
| Author;
KONISHI ASAKO
(Otsu Red Cross Hospital, JPN)
AOSHIMA TOSHIYUKI
(Otsu Red Cross Hospital, JPN)
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Journal Title;Skin Research
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Journal Code:Z0014C
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ISSN:1347-1813
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VOL.5;NO.1;PAGE.49-52(2006)
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| Figure&Table&Reference;FIG.8, REF.8 |
| Pub. Country;Japan |
| Language;Japanese |
| Abstract;Juvenile hyaline fibromatosis is a rare autosomal recessive disorder characterized by the papulonodular skin lesions, joint contractures and bone lesions clinically and deposition of hyaline-like materials in various tissues histopathologically. We report a case of 44-year-old man who had typical clinical and histopathologic features of this disease. Symptom started at the age of 6 as multiple skin-colored hard tumors on his back, scalp, paranasal fold, gingiva and extensor surface of knees and elbows. Joint contracture progressed gradually with growing and increasing in number of tumors, which has made him completely bedridden for these several years. Skin biopsy showed deposition of much amount of amorphous, PAS positive hyaline-like materials in the dermis and subcutaneous tissue in which blood vessels kept their shape. (author abst.) |
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