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KMID : 0363219940320061090
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Korean Journal of Dermatology
1994 Volume.32 No. 6 p.1090 ~ p.1094
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A Case of Hunter Syndrome with Caracteristic Skin Lesions
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Abstract
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We report a typical case of Hunter's syndrome in a 9 year old boy, who presented with firm skin colored nodules that coalesce to form a reticular pattern on a symmetrical area between the angles of the scapulas, which is regarded as the
pathognomonic
cutaneous markers for Hunter's syndrome. He also showed growth retardation, clear corneas, hepatomegaly, attention deficit and mild mental deterioration. The skin biopsy specimen taken from a typical nodule shows loosely arranged collagen fibers
with
masive mucinous material which stains positively with alcian blue at both pH 2.5 and 0.5, metachromatic granules within fibroblast stained with toluidine blue. (Kor J Dermatol 1994 ; 32(6) : 1090~1094)
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