경피골막증에 동반된 크론병

Abstract

Pachydermoperiostosis is a rare hereditary syndrome characterized by finger clubbing, periosteal change, pachydermia and autonomic nervous system symptoms such as facial flushing and hy perhidrosis. This syndrome is known to be associated with a variety of diseases such as cranial suture defect, female escuchen, bone marrow failure, hypertrophic gastropathy and Crohn' s disease. Crohn' s disease is a chronic idiopathic inflammatory disease of the intestine and frequently presents many kinds of extraintestinal manifestations including finger clubbing. Recently, Compton et al. reported 3 patients with Crohn' s disease associated with pachydermoperiostosis in a family as a new syndrome Recently, we have also experienced 3 patients with pachydermoperiostosis in a family. One of them developed Crohn' s disease at 45 years of age. The elder sister of the proband had protein loosing enteropathy without a significant morphological change of gastrointestinal tract. The younger brother had experienced repeated gastrointestinal bleeding caused by multiple ileal ulcer.