{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T07:43:21Z","timestamp":1792827801323,"version":"4.5.0"},"reference-count":43,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2019,3,5]],"date-time":"2019-03-05T00:00:00Z","timestamp":1551744000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acad Dermatol Venereol"],"published-print":{"date-parts":[[2019,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Ecthyma gangrenosum (<jats:styled-content style=\"fixed-case\">EG<\/jats:styled-content>) is characterized by the occurrence of erythematous, violaceous or haemorrhagic macules and\/or vesicles, often evolving into necrotic ulcers, with a central grey\u2013black eschar. It is a rare skin condition, usually occurring in immunocompromised patients suffering from bacterial sepsis caused by <jats:italic>Pseudomonas aeruginosa<\/jats:italic>. However, seemingly healthy children have been diagnosed with this skin disease as well.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>We report the workup of a case of vulvar <jats:styled-content style=\"fixed-case\">EG<\/jats:styled-content> caused by <jats:italic>P.\u00a0aeruginosa<\/jats:italic> in a toddler, which led to a diagnosis of an underlying neutropenia. Moreover, we provide a brief literature review on those cases of <jats:styled-content style=\"fixed-case\">EG<\/jats:styled-content> where an underlying primary immunodeficiency, neutropenia in particular, was eventually diagnosed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A one\u2010and\u2010a\u2010half\u2010year\u2010old girl presented with a history of recurrent (respiratory) infections and the sporadic occurrence of purpuric, vulvar ulcers. Workup consisted of microbiological and haematological investigations, including repeated blood analyses.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Bacterial swabs from the vulvar ulcers showed the growth of <jats:italic>P.\u00a0aeruginosa<\/jats:italic>. No concomitant sepsis was present, but laboratory investigations pointed towards a cyclic neutropenia, coinciding with the occurrence of the <jats:styled-content style=\"fixed-case\">EG<\/jats:styled-content> lesions. Topical gentamicin ointment allowed the skin lesions to heal faster. Following the administration of granulocyte colony\u2010stimulating factor (G\u2010CSF), the girl experienced less infections in general and had no recurrence of <jats:styled-content style=\"fixed-case\">EG<\/jats:styled-content> lesions in particular. Treatment with G\u2010CSF could eventually be stopped, and the neutropenia, ultimately transient in nature, completely resolved.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Children presenting with (anogenital) <jats:styled-content style=\"fixed-case\">EG<\/jats:styled-content> should always alert a physician to consider a potentially underlying immunodeficiency, neutropenia in particular.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jdv.15420","type":"journal-article","created":{"date-parts":[[2019,1,11]],"date-time":"2019-01-11T16:49:46Z","timestamp":1547225386000},"page":"781-785","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Juvenile ecthyma gangrenosum caused by <i>Pseudomonas aeruginosa<\/i> revealing an underlying neutropenia: case report and review of the 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