中性粒细胞性皮肤病:甜综合征和坏疽性脓皮病。文献回顾和自己的临床观察

Y. Yehudina, S. Trypilka, I. Golovach
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摘要

中性粒细胞性皮肤病是一种异质性的炎症性皮肤病,具有独特的临床特征,但在组织病理学检查中表现为无菌的,主要是中性粒细胞浸润。中性粒细胞性皮肤病包括甜综合征、坏疽性脓皮病、白塞病、中性粒细胞性荨麻疹、角膜下脓疱性皮肤病、掌跖性脓疱病、异位性肢端皮炎、急性广泛性脓疱病、婴儿脓疱病等。与这些疾病相关的皮肤病变形态是异质的,这使得诊断具有挑战性。此外,对患者进行彻底的临床评估是必要的,以排除类似这些疾病的疾病,并诊断潜在的伴随感染、炎症和肿瘤过程。虽然一些中性粒细胞性皮肤病可能会自发消失,但大多数需要治疗才能缓解。诊断和治疗的延误可能导致这些患者的严重发病率甚至死亡率。糖皮质激素是治疗中性粒细胞性皮肤病的首选药物。在它们无效的情况下,根据皮肤病变的严重程度、疾病的慢性、是否存在全身病变和难治性,可以使用其他生物疾病缓解抗风湿药物。本文在回顾现代文献和本人临床观察的基础上,对甜证和坏疽性脓皮病的流行病学、发病机制、临床特征、诊断征象和现代治疗方法进行了综述。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Neutrophilic dermatoses: Sweet syndrome and gangrenous pyoderma. Literature review and own clinical observations
Neutrophilic dermatoses are a heterogeneous group of inflammatory skin diseases that have unique clinical features, but are combined by the presence of a sterile, predominantly neutrophilic infiltrate at histopathological examination. Neutrophilic dermatoses include Sweet syndrome, gangrenous pyoderma, Behcet’s disease, neutrophilic urticaria, subcorneal pustular dermatosis, palmoplantaris pustulosis, acrodermatitis Allopo, acute generalized exentematous pustulosis, infant pustulosis, etc. The morphology of skin lesions associated with these diseases is heterogeneous, which makes diagnosis challenging. In addition, a thorough clinical evaluation of patients is necessary to exclude diseases that mimic these disorders and to diagnose potential concomitant infectious, inflammatory, and tumor processes. While some neutrophilic dermatoses may disappear spontaneously, most require treatment to achieve remission. A delay in diagnosis and treatment can lead to significant morbidity and even mortality in these patients. Glucocorticoids are the drugs of choice for the treatment of neutrophilic dermatoses. In case of their ineffectiveness, other biological disease-modifying antirheumatic drugs may be administered depending on the severity of the skin lesion, chronicity of the disease, the presence of systemic lesions, and refractory status. This work is devoted to epidemiology, etiopathogenesis, clinical features, diagnostic signs and modern methods of treating Sweet syndrome and gangrenous pyoderma based on a review of modern literature and our own clinical observations.
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