Vongsachang Hursuong, Chiou Carolina A, Azad Amee D, Lin Lisa Y, Yoon Michael K, Lefebvre Daniel R, Stagner Anna M
Department of Ophthalmology, Ophthalmic Plastic Surgery, Massachusetts Eye and Ear, Harvard Medical School, Boston, MA, USA.
David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye and Ear, Harvard Medical School, Boston, MA, USA.
Am J Ophthalmol Case Rep. 2024 Mar 2;34:102033. doi: 10.1016/j.ajoc.2024.102033. eCollection 2024 Jun.
Two cases are described of necrotizing Sweet syndrome (nSS), a rare variant of acute febrile neutrophilic dermatosis that mimics necrotizing soft tissue infections.
A 74-year-old female with myelodysplastic syndrome (MDS) presented with isolated periorbital nSS that closely mimicked necrotizing fasciitis (NF); she displayed pathergy to debridement, was exquisitely responsive to corticosteroids, and underwent successful first-stage reconstruction of the eyelid with full-thickness skin grafting. A second 40-year-old female patient with relapsed acute myelogenous leukemia (AML) presented with multifocal nSS most prominently involving the eyelid. Positive herpes zoster virus (HSV) PCR and bacterial superinfection complicated the diagnosis. She improved with chemotherapy for AML and corticosteroid therapy.
nSS is rare and a high level of clinical suspicion as well as an understanding of its distinguishing features is necessary to avoid undue morbidity. Identification of pathergy, histopathology, microbiology, and clinical context are critical to avoid misdiagnosis of infection.
描述两例坏死性Sweet综合征（nSS），这是一种急性发热性嗜中性皮病的罕见变体，可模仿坏死性软组织感染。
一名74岁患有骨髓增生异常综合征（MDS）的女性出现孤立性眶周nSS，酷似坏死性筋膜炎（NF）；她对清创术表现出同形反应，对皮质类固醇极为敏感，并通过全厚皮片移植成功进行了眼睑一期重建。第二名40岁复发性急性髓性白血病（AML）女性患者出现多灶性nSS，最显著累及眼睑。疱疹带状疱疹病毒（HSV）PCR阳性和细菌二重感染使诊断复杂化。她通过AML化疗和皮质类固醇治疗后病情改善。
nSS罕见，需要高度的临床怀疑以及对其特征的了解，以避免不必要的发病率。识别同形反应、组织病理学、微生物学和临床背景对于避免感染的误诊至关重要。
