La Presse medicale Pub Date : 2020-02-10 DOI:10.32388/9dqhj7
E. Mahé, V. Descamps, S. Bélaïch, B. Cricks
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摘要

一般特征:罕见的皮肤溃疡,坏疽性脓皮病是中性粒细胞皮炎的一种,其特征是成熟的中性粒细胞多核皮肤浸润丰富。原发病变是位于炎症基础上的脓疱;非常痛苦,它会迅速溃烂。病变迅速扩展至直径超过10cm,边界规则、尖锐,周围有肉卷,内部有化脓通道,红色颗粒状表面常被脓疱覆盖。人们对这种疾病所知甚少。合并症:坏疽性脓皮病合并其他疾病的病例超过50%,必须系统查找。这些可能是消化性的,本质上是炎症性的小肠结肠炎,经常发展为肠周溃疡,风湿性疾病,特别是类风湿关节炎,血液学疾病(良性单克隆γ病,慢性髓系血液病)。从临床的角度来看:没有具体的检查。在所有病例中都应进行皮肤活检,特别是为了消除溃疡的其他原因。由于随后可能会发现伴随疾病,因此即使在疾病治愈后,也必须重新进行临床旁调查。没有明确的治疗方法:如果可以治愈,对病因的治疗可能足以使病变消退。必须进行局部治疗以引起萌芽,从而避免感染。在进行性和广泛性形式中,需要全身治疗,特别是大剂量皮质类固醇。先验地排除手术。
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[Pyoderma gangrenosum].
GENERAL CHARACTERISTICS: Rare cause of cutaneous ulceration, pyoderma gangrenosum is among the group of neutrophilic dermatites characterized by the richness of the mature neutrophilic polynuclear dermal infiltrate. The primary lesion is a pustule sitting on an inflammatory base; extremely painful, it rapidly ulcerates. The lesion rapidly extends to more than 10 cm in diameter, has a regular, sharp border and a peripheral roll of flesh exhibiting purulent channels on the inside and a red granulous surface often covered with a pustular coating. Little is known of this disease. CONCOMITANT AFFECTIONS: In more than 50% of cases, pyoderma gangrenosum is associated with other diseases, which must be systematically searched for. These may be digestive, essentially inflammatory enterocolitis with frequent development of peristomal ulceration, rheumatismal affections notably rheumatoid arthritis, hematological affections (benign monoclonal gammapathy, chronic myeloid hemopathy). FROM A PARACLINICAL POINT OF VIEW: There are no specific examinations. A cutaneous biopsy should be performed in all cases, notably to eliminate other causes of ulceration. Since concomitant disease can be subsequently revealed, it is essential to renew the paraclinical investigations, even after the disease has healed. NO CODIFIED TREATMENT: Treatment of the cause, if it can be cured, may be sufficient to permit regression of the lesions. Local treatments to provoke budding and hence avoid surinfection are mandatory. In the progressive and extensive forms, systemic treatment, notably high dose corticosteroids, is indicated. Surgery, a priori, is excluded.
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[Pyoderma gangrenosum]. [Hypocomplementemic urticarial vasculitis]. [Trisomy 13]. [Osteogenesis imperfecta]. [Hypersomnia].
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