Document Type : case report


1 Eye Research Center, Mashhad University of Medical Sciences, Mashhad, Iran

2 Assistant Professor of Ophthalmology Department of Ophthalmology, School of Medicine Khatam Al Anbiya Hospital Mashhad University of Medical Sciences

3 Department of Pediatrics, Mashhad University of Medical Sciences, Mashhad, Iran


Purpose: This article aimed to report an infant case of congenital neutropenia (CN) with periorbital ecthyma gangrenosum (EG) due to pseudomonas aeruginosa septicemia.
Case report: A previously healthy six-month-old male infant with fever, diarrhea, poor feeding, pancytopenia, periorbital swelling, and eyelid ulcers in the left eye was admitted in pediatric intensive care unit (PICU). He had positive blood and eyelid wound cultures for pseudomonas aeruginosa. He was treated with broad spectrum intravenous antipseudomonal antibiotics and debridement of periorbital lesions and discharged after 18 days with a stable condition. Adequate hematologic workup for pancytopenia revealed the diagnosis of CN.
Conclusion: There is a possibility that EG be developed in patients with immunodeficiency as preseptal cellulitis with isolated typical lesions, and this should be considered in the treatment.


  1. Shah M, Crane JS. Ecthyma Gangrenosum. (Updated 2021 Jul 18). In: StatPearls (Internet). Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from:
  2. Vaiman M, Lazarovitch T, Heller L, Lotan G. Ecthyma gangrenosum and ecthyma-like lesions: review article. Eur J Clin Microbiol Infect Dis. 2015 Apr; 34(4):633-9. doi: 10.1007/s10096-014-2277-6. Epub 2014 Nov 19. PMID: 25407372.
  3. Lin Q, Hu B, Shi J, Shi W, Cao W, Li L. Eyelid and periorbital ecthyma gangrenosum due to Pseudomonas aeruginosa in an infant. Pediatr Investig. 2017; 1(1):47-49. Published 2017 Dec 27. doi:10.1002/ped4.12005.
  4. Henshaw EB, Ibanga AA, Obaji DP. Fatal Oculocutaneous Ecthyma Gangrenosum in Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome: Case Report and Review of the Literature. J Glob Infect Dis. 2019; 11(1):43-46. doi:10.4103/jgid.jgid_54_18.
  5. Steinkogler FJ, Huber-Spitzy V. Necrotizing destruction of the ocular adnexa by Pseudomonas aeruginosa. J Craniomaxillofac Surg. 1988 Jan; 16(1):28-30. doi: 10.1016/s1010-5182(88)80010-6. PMID: 3123523.
  6. Kim HJ, Grossniklaus HE, Wojno TH. Periorbital ecthyma gangrenosum: a case report and review of the literature. Ophthalmic Plast Reconstr Surg. 2014 Sep-Oct; 30(5):e125-8. doi: 10.1097/IOP.0b013e3182a6504a. PMID: 24317102.
  7. Ghosheh FR, Kathuria SS. Bilateral periorbital ecthyma gangrenosum. Ophthalmic Plast Reconstr Surg. 2006 Nov-Dec; 22(6):492-3. doi: 10.1097/01.iop.0000248990.71690.a2. PMID: 17117116.
  8. Watson A, Sloan B. Ecthyma gangrenosum arising from Pseudomonas aeruginosa dacryocystitis. Clin Exp Ophthalmol. 2003 Aug; 31(4):366-8. doi: 10.1046/j.1442-9071.2003.00675.x. PMID: 12880468.
  9. Donadieu J, Fenneteau O, Beaupain B, Mahlaoui N, Chantelot CB. Congenital neutropenia: diagnosis, molecular bases and patient management. Orphanet J Rare Dis. 2011 May 19; 6:26. doi: 10.1186/1750-1172-6-26. PMID: 21595885; PMCID: PMC3127744.
  10. Kimyai-Asadi A, Tausk FA, Nousari HC. Ecthyma secondary to herpes simplex virus infection. Clin Infect Dis. 1999 Aug; 29(2):454-5. doi: 10.1086/520241. PMID: 10476767.
  11. Bassetti M, Vena A, Croxatto A, Righi E, Guery B. How to manage Pseudomonas aeruginosa infections. Drugs Context. 2018 May 29; 7:212527. doi: 10.7573/dic.212527. PMID: 29872449; PMCID: PMC5978525.
  12. Klein C. Check for Updates Hematology Am Soc Hematol Educ Program (2009) 2009 (1): 344–350.