Todd Nicole, Boucher Julia E, Bassal Mylène, Dumont Tania, Fleming Nathalie
Department of Obstetrics and Gynecology, University of British Columbia, Vancouver, British Columbia, Canada.
Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada.
J Pediatr Adolesc Gynecol. 2018 Dec;31(6):625-628. doi: 10.1016/j.jpag.2018.05.007. Epub 2018 Jun 6.
An 18-month-old female toddler presented with severe vulvar ulcers and pancytopenia with investigations revealing Pseudomonas aeruginosa bacteremia.
A previously healthy 18-month-old female toddler presented with 6 days of fevers, vulvar rash, and ulcers. Vulvar cultures showed Staphylococcus aureus and P aeruginosa. Bloodwork showed pancytopenia and P aeruginosa bacteremia. She started receiving broad-spectrum antibiotics. Bone marrow aspirate revealed a hypocellular marrow with erythroid dysplasia. Vulvar ulcers progressed rapidly, therefore magnetic resonance imaging was performed to rule out necrotizing fasciitis. She was diagnosed with ecthyma gangrenosum (EG). Three months after initial presentation, she was diagnosed with precursor B-cell acute lymphoblastic leukemia.
This case highlights that health care providers should suspect EG when severe vulvar ulcers are present with P aeruginosa infection and neutropenia. Because EG poses significant morbidity and mortality, its presence should prompt aggressive antimicrobial therapy and mobilization of a multidisciplinary team to initiate workup for an underlying immunodeficiency syndrome or malignancy. This case also illustrates that surgical debridement might be avoided in certain patients with EG as long as meticulous wound care and close monitoring with a multidisciplinary team are in place.
一名18个月大的女幼儿出现严重的外阴溃疡和全血细胞减少，检查发现铜绿假单胞菌败血症。
一名此前健康的18个月大女幼儿出现发热、外阴皮疹和溃疡6天。外阴培养显示金黄色葡萄球菌和铜绿假单胞菌。血液检查显示全血细胞减少和铜绿假单胞菌败血症。她开始接受广谱抗生素治疗。骨髓穿刺显示骨髓细胞减少伴红系发育异常。外阴溃疡迅速进展，因此进行了磁共振成像以排除坏死性筋膜炎。她被诊断为坏疽性脓皮病（EG）。初次就诊3个月后，她被诊断为前体B细胞急性淋巴细胞白血病。
本病例强调，当出现严重外阴溃疡并伴有铜绿假单胞菌感染和中性粒细胞减少时，医疗保健人员应怀疑坏疽性脓皮病。由于坏疽性脓皮病会导致严重的发病率和死亡率，其出现应促使积极的抗菌治疗，并动员多学科团队对潜在的免疫缺陷综合征或恶性肿瘤进行检查。本病例还表明，对于某些坏疽性脓皮病患者，只要有细致的伤口护理并由多学科团队密切监测，就可以避免手术清创。
