구강내 발생한 심상성 천포창 환자의 치험례
Oral pemphigus vulgaris
김일규, 최진웅, 김루시아, 양정은, 장재원, 사시카라 바라라만,
소속 상세정보
김일규 ( Kim Il-Kyu ) - 인하대학교 의과대학 치과학교실
최진웅 ( Choi Jin-Ung ) - 인하대학교 의과대학 치과학교실
김루시아 ( Kim Lucia ) - 인하대학교 의과대학 병리학교실
양정은 ( Yang Jung-Eun ) - 인하대학교 의과대학 치과학교실
장재원 ( Jang Jae-Won ) - 인하대학교 의과대학 치과학교실
사시카라 바라라만 ( Sasikala Balaraman ) - 인하대학교 의과대학 치과학교실
KMID : 0360120090310050414
Abstract
Pemphigus vulgaris is a chronic autoimmune intraepithelial blistering disease with oral mucosal manifestations that very often precede the skin lesions. The vesicles or bullae are produced by an acantholytic process, detachment of differentiating keratinocytes from one another in the epithelial stratum spino sum or spinous cell layer. The pathogenesis of this disease is initially manifested by IgG(mainly) binding to desmosome(desmoglein 3 or 1) in the intercellular spaces of epithelium. This autoantibody binding caused the release of a plasminogen activator(a proteolytic enzyme) from keratinocytes. This ultimately results in cell to cell separation. The mainstay therapy of pemphigus vulgaris is systemic corticosteroids and immunosuppressive agents to eliminate the pathogenic autoantibodies from circulation. A 41-year old woman presented with a 1.5 year history of oral ulceration. There were no lesions on the skin or other mucosal sites. Histology and immunostaining were consistent with pemphigus vulgaris. Control of oral ulceration and normal oral function were achieved after systemic corticosteroids and immunosuppressive agents were instituted.
키워드
Pemphigus vulgaris;Oral mucosa
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