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[发热性溃疡性坏死性穆查-哈伯曼病]

[Febrile ulceronecrotic Mucha-Habermann disease].

作者信息

Dupin A, Bosset D, Atger L, Chevallier B, Saiag P, Benoist G

机构信息

Service de pédiatrie générale, CHU Ambroise-Paré, AP-HP, 9, avenue Charles-de-Gaulle, 92100 Boulogne-Billancourt, France.

Service de dermatologie, CHU Ambroise-Paré, AP-HP, 9, avenue Charles-de-Gaulle, 92100 Boulogne-Billancourt, France; UFR des sciences de la santé Simone-Veil, université de Versailles St. Quentin, 78180 Montigny-le-Bretonneux, France.

出版信息

Arch Pediatr. 2016 Jan;23(1):82-5. doi: 10.1016/j.arcped.2015.09.029. Epub 2015 Nov 6.

Abstract

Pityriasis lichenoides et varioliformis acuta (PLEVA) is an inflammatory skin disease that is unknown to pediatricians. The ulceronecrotic febrile form is a rare and potentially lethal variant. We report the case of a 7-year-old boy with a papulovesicular eruption lasting for 4 weeks, secondarily associated with ulcers and necrotic crusts, fever, and systemic signs. After exploring infectious causes such as chickenpox, we discussed and confirmed PLEVA with histological analysis. Systemic steroids and methotrexate improved the symptoms. It is necessary to mention PLEVA in case of prolonged papulovesicular eruption. Prompt diagnosis allows appropriate treatment, although there is no consensus on therapeutic guidelines.

摘要

急性痘疮样苔藓样糠疹(PLEVA)是一种儿科医生并不了解的炎症性皮肤病。溃疡性坏死发热型是一种罕见且可能致命的变体。我们报告了一名7岁男孩的病例,其丘疹水疱性皮疹持续4周,继发于溃疡、坏死痂皮、发热和全身症状。在排查了水痘等感染性病因后,我们通过组织学分析讨论并确诊为PLEVA。全身性类固醇和甲氨蝶呤改善了症状。对于持续时间较长的丘疹水疱性皮疹,有必要考虑PLEVA。尽管治疗指南尚无共识,但及时诊断有助于进行适当治疗。

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