Submucosal Fibrosis with Severe IgK Deposition in Behcet's Syndrome

Park, Young Wook; Kim, Yeon Sook; Lee, Suk Keun

Abstract

A case of Behcet's syndrome found in a 22 years old male was is reported along with the histological and immunohistochemical studiesfindings. He The patient had had received prolonged medical therapy of anti-inflammatory agents for the conjunctival ulcer, genital ulcer, oral ulcer, and intestinal ulcers since the first onset of the Behcet's syndrome about approximately 7 years agoearlier. Recently, he felt the athickening of both corners of his lip causing that caused mouth opening difficultydifficulties. A plastic rReconstructive surgery was performed to enlarge the size of the oral orifice by multiple Z-plasty incisions, and finallywhich resulted in proper enlargement of the circumferential length of the lip. During the operation, a scar-like thick fibrous tissue was obtained and examined pathologically. In theThe microscopic observation the revealed the submucosal lesion was to be diffusely fibrosed with the a distribution of sclerotic collagen bundles. Particularly, sSeveral foci of collagen degeneration were found observed in the deep connective tissue, and the degenerating collagen bundles were gradually lost their fibrillar appearance. In the immunohistochemical observations, the foci of collagen degeneration was were strongly positive for IgK, but almost negative for TNFalpha lysozyme, and MMP-3. Taken together, it was presumed that tThe submucosal fibrosis was presumed to have firstly presented in this study was probably beeninduced by the prolonged anti-inflammatory therapy, which may inhibit the removal of sclerosed collagen bundles by the cell-mediated immunity and proteolytic digestion of macrophages, and that it was secondarily aggravated by the deposition of immunoglobulins derived from an autoimmune origin. Therefore, even after the successful plastic surgery of the lip to ameliorate the mouth opening difficulty, the recurrent submucosal fibrosis of lip should be carefully managed in the follow-up treatment.