Bonotto G, Tonetto F, Baraglia E, Battistioli M, Fabi F, Arrigoni M, Spolaore R
Divisione di Chirurgia Generale, USSL 11-Oderzo, Treviso.
Minerva Chir. 1992 Oct 31;47(20):1641-5.
Pseudoxanthoma Elasticum (PXE) is a rare inherited elastic tissue disorder with degeneration of elastic fibres involving mainly skin, eyes and the cardiovascular system. Upper gastro-intestinal hemorrhage occurs in 13% of cases and is often resistant to non-surgical methods of treatment. One case report involving recurrent gastric hemorrhage in a young patient with PXE and treated with total gastrectomy is presented. Diagnosis was made, after surgery, by biopsy of skin lesions of the neck. The characteristics of PXE are reviewed and the case is discussed.
弹性假黄瘤(PXE)是一种罕见的遗传性弹性组织疾病,其弹性纤维变性主要累及皮肤、眼睛和心血管系统。13%的病例会发生上消化道出血,且通常对非手术治疗方法耐药。本文报告一例年轻的弹性假黄瘤患者反复发生胃出血并接受全胃切除术的病例。术后通过颈部皮肤病变活检作出诊断。本文对弹性假黄瘤的特征进行了综述并讨论了该病例。