[伴有肝脾定位及单克隆丙种球蛋白病的皮肤坏疽性脓皮病。病例报告]

[Cutaneous pyoderma gangrenosum with hepatosplenic localization and monoclonal gammapathy. A case report].

作者信息

Delbrel X, Germain P, Fach J, Etienne G, Beylot C, Parrens M, De Mascarel A, Le Bras M, Longy-Boursier M

机构信息

Service de Médecine Interne et de Maladies Tropicales, Hôpital Saint-André, 1, rue Jean-Burguet, 33075 Bordeaux Cedex.

出版信息

Ann Med Interne (Paris). 2001 Feb;152(1):65-7.

PMID:11240430

Abstract

Pyoderma gangrenosum is an ulcerative disease of the skin. The histopathological lesions are nonspecific, characterized by a diffuse neutrophilic infiltrate in the dermis. Pyoderma gangrenosum is associated with inflammatory, digestive or articular disease, or acute or chronic hemotology disorders in 50% of the cases, more rarely with monoclonal gammapathy. A visceral localization of pyoderma gangrenosum is rare, simulating a systemic disease or an underlying neoplasia. We report a case of cutaneous pyoderma gangrenosum with splenic and hepatic localizatios associated with an IgG monoclonal gammapathy. We emphasize the efficacy of immunosuppressor treatment and the importance of long-term monitoring of these patients.

摘要

坏疽性脓皮病是一种皮肤溃疡性疾病。其组织病理学病变是非特异性的，特征为真皮内弥漫性中性粒细胞浸润。50%的坏疽性脓皮病病例与炎症性、消化系统或关节疾病，或急性或慢性血液系统疾病有关，较少与单克隆丙种球蛋白病相关。坏疽性脓皮病的内脏定位罕见，可模拟全身性疾病或潜在的肿瘤形成。我们报告一例伴有脾脏和肝脏定位且与IgG单克隆丙种球蛋白病相关的皮肤坏疽性脓皮病病例。我们强调免疫抑制剂治疗的有效性以及对这些患者进行长期监测的重要性。