家族性厚皮性骨膜病伴蛋白丢失性肠病。

Familial pachydermoperiostosis in association with protein-losing enteropathy.

作者信息

Sethuraman G, Malhotra A K, Khaitan B K, Sharma V K, Kumar R, Makharia G K, Vinod B N, Sharma S K, Goswami R, Bandhu S

机构信息

Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Clin Exp Dermatol. 2006 Jul;31(4):531-4. doi: 10.1111/j.1365-2230.2006.02138.x.

DOI:10.1111/j.1365-2230.2006.02138.x

PMID:16716156

Abstract

In this report we describe a rare association of pachydermoperiostosis with protein-losing enteropathy (PLE) in a family of three brothers. The first brother had the complete form of pachydermoperiostosis along with PLE. The second brother had the 'forme fruste' of pachydermoperiostosis, with minimal skin changes, bony abnormalities and PLE, which was due to intestinal lymphangiectasia. The third brother had an incomplete form of pachydermoperiostosis without evidence of PLE. To our knowledge, the association of pachydermoperiostosis with PLE due to intestinal lymphangiectasia has not been reported previously.

摘要

在本报告中，我们描述了一个三兄弟家庭中厚皮性骨膜病与蛋白丢失性肠病（PLE）的罕见关联。大哥患有完全型厚皮性骨膜病及PLE。二哥患有厚皮性骨膜病的“顿挫型”，皮肤变化、骨骼异常及PLE均很轻微，这是由肠道淋巴管扩张引起的。三弟患有不完全型厚皮性骨膜病，无PLE证据。据我们所知，此前尚未报道过厚皮性骨膜病与因肠道淋巴管扩张导致的PLE之间的关联。