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 Table of Contents  
Year : 2023  |  Volume : 3  |  Issue : 1  |  Page : 12-14

Severe ocular involvement in ecthyma gangrenosum

1 Department of Ophthalmology, Atal Bihari Vajpayee Institute of Medical Sciences and Dr Ram Manohar Lohia Hospital (ABVIMS), New Delhi, India
2 Consultant Cornea, Cataract and Refractive Surgeon, Kekan Hospital, Gangapur Road, Nasik, Maharashtra, India

Date of Submission28-Apr-2022
Date of Acceptance17-Aug-2022
Date of Web Publication20-Jan-2023

Correspondence Address:
Mitali Kekan
Kekan Hospital, Gangapur Road, Nasik - 422 002, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1076_22

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Ecthyma Gangrenosum (EG) is a cutaneous infection occurring mainly in immunocompromised patients with very rare ocular involvement. We report a rare case of an immunocompetent, 16 year old female with bilateral pseudomembranous conjunctivitis preceded by dermatological lesions. Timely intervention and appropriate treatment helped salvage globe in both eyes with improvement of vision in right eye. Only 2 cases have been reported till date of Ecthyma Gangrenosum with ocular involvement. In both the case reports, the globe could not be salvaged.

Keywords: Descemetocele, ecthyma gangrenosum, ocular involvement, perforation

How to cite this article:
Jain S, Kekan M, Mammel A, Pathak A. Severe ocular involvement in ecthyma gangrenosum. Indian J Ophthalmol Case Rep 2023;3:12-4

How to cite this URL:
Jain S, Kekan M, Mammel A, Pathak A. Severe ocular involvement in ecthyma gangrenosum. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 2];3:12-4. Available from: https://www.ijoreports.in/text.asp?2023/3/1/12/368122

Ecthyma gangrenosum (EG) is a cutaneous infection associated with Pseudomonas septicemia in immunocompromised and critically ill patients.[1] The cutaneous findings are characterized by small indurated papulovesicles progressing rapidly into necrotic ulcers with surrounding erythema and central black eschar.[2] The periorbital and ocular involvement are rare in ecthyma gangrenosum.

  Case Report Top

A 16-year-old female presented with swelling of both eyes associated with watering and photophobia for 14 days and multiple reddish hyperpigmented lesions over the scalp which preceded the lid swelling by 25 days. The skin lesions were 2 × 1 cm in size, multiple in number, located over the scalp and neck area. The lesions had an ulcerative base with erythema.

Ocular examination showed that visual acuity in both eyes was perception of light. Lid and adnexa showed tense edema with erythema. Copious mucopurulent discharge with pseudomembranes over the palpebral conjunctiva was noted in both eyes.

Anterior segment examination showed diffuse corneal haze in the right eye with 2 to 3 o'clock hours of limbal ischemia in the inferonasal area. A dense anterior chamber reaction with membranes was noted in the pupillary area. The left eye had a central corneal epithelial defect of 5 × 2 mm. No infiltrate was appreciated. A grade 4 anterior chamber reaction was noted. Posterior segment examination in both eyes could not be done due to hazy media. Imaging revealed anechoic posterior segment in both eyes.

The patient was started on topical moxifloxacin drops hourly in view of conjunctivitis, and topical steroids were withheld in view of suspected infective etiology. Oral steroids were started in the form of tab. prednisolone 40 mg OD for dense anterior chamber reaction.

Dermatological reference was done and the patient was advised topical mupirocin ointment for suspected scalp ecthyma. On day 3, the patient reported worsening of symptoms. Skin lesions had increased in both size and number. A change in morphology was noted in the form of an overlying necrotic eschar along with induration [Figure 1]a.
Figure 1: (a) Skin lesions on day 3 of presentation. (b) Healed skin lesions at 2 months

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The right eye had progressed to scleral ischemia in the inferonasal quadrant. A 3-mm hypopyon was noted in the anterior chamber. In the left eye, the patient denied perception of light and an infiltration had developed surrounding the epithelial defect. Corneal scraping was taken and sent for microbiology examination and culture report.

A repeat dermatology reference was done and based on typical lesion morphology, diagnosis of ecthyma gangrenosum was made and oral steroids were stopped.

Baseline blood investigations including viral markers were negative. Serum C-reactive protein (CRP) was raised and serum procalcitonin—a marker of systemic inflammation and sepsis—was positive (0.5–2 ng/ml). None of the materials taken from various sites of the lesion including the corneal scrapings showed any microorganisms. Edge biopsy from skin lesion showed dense inflammation in the subepithelial tissue. However, no viral inclusions, fungal elements, or bacteria were seen.

In view of strong clinical suspicion of ecthyma gangrenosum, the patient was started on inj. piperacillin tazobactem 4.5 gm IV TDS along with inj. linezolid 600 mg BD for 14 days. Tablet fluconazole 100 mg BD for 7 days was given to cover fungal elements. Topical antibiotic therapy was stepped up in the form of tobramycin to cover gram-negative organisms. Daily dressing of the skin lesions was done. During the course of the stay, the patient developed an episode of shock for which she was shifted to the intensive care unit (ICU) and managed appropriately.

On day 7, while in the ICU the right eye developed scleral thinning along with severe corneal thinning leading to descemetocele [Figure 2]a. The left eye was noted to have developed a central corneal perforation with iris incarceration in the wound. Perforation was managed with tissue adhesive and bandage contact lens (TA-BCL) [Figure 2]b. The patient was shifted to topical hourly fortified eye drops (fortified cephazoline 5% hourly, fortified tobramycin 1.3% hourly). Steroids were withheld. In subsequent follow-up, gradual healing was noted.
Figure 2: Day 7 (a) RE: scleral thinning with central descemetocele. (b) LE: central corneal perforation with iris incarceration

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At two-month follow-up, the skin lesions had healed completely [Figure 1]b. The right eye developed total conjunctivalization of the cornea with pannus and a central descemetocele [Figure 3]a. Anterior segment optical coherence tomography (AS-OCT) of the right eye showed thinned out cornea with pannus adherent to the iris. The left eye healed with central vascularized scarring [Figure 3]b. Topical steroids were added in the left eye at this stage. At 4-month follow-up, a grade 4 symblepharon with ankyloblepharon involving the puncta was noted [Figure 4]a. Vision in the right eye had improved to hand movement close to face with accurate projection of rays. The globe integrity of the left eye was maintained with central adherent leucoma and vascularization. Vision remained PL negative [Figure 4]b. Visual-evoked potential in the right eye showed prolonged p100 latency and no response from the left eye.
Figure 3: 2 months follow-up. (a) RE: conjunctivalization of the cornea with descemetocele. (b) LE: perforation sealed with TA-BCL

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Figure 4: Follow up at 4 months (a) RE; grade 4 symblepharon with ankyloblepharon with punctal involvement. (b) LE: central scarring with vascularization

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  Discussion Top

Ecthyma gangrenosum (EG) is a rare cutaneous manifestation of a systemic infection. Microbial invasion of the arteries in the dermis and subcutaneous tissue produces a necrotizing vasculitis.[3] A characteristic lesion due to EG is a hemorrhagic pustule evolving into a necrotic ulcer. The most common pathogen implicated is Pseudomonas aeruginosa.[4] However, other microorganisms like bacteria and fungi have been implicated.[5] The most common site of EG is the gluteal and perineal region. Facial involvement is reported to occur in 6% of cases, periocular involvement is rare, and ocular involvement is still rarer.[5],[6] Only two cases of ocular ecthyma have been reported till date, both presenting with panophthalmitis. The involved eye had to be eviscerated in one case and exenterated in the other.[7],[8]

Our patient was immunocompetent without any systemic comorbidities. Even though the cultures and histopathological tests were negative, review of literature and typical lesion morphology helped make the diagnosis.

  Conclusion Top

Timely institution of appropriate systemic antibiotics in our cases of ecthyma gangrenosum helped in healing scalp lesions and preventing further progression of eye disease, thereby improving the visual prognosis in the right eye and preserving the globe in both eyes.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Chang AY, Carlos CA, Schuster M, Xu X, Rosenbach M. Nonpseudomonal ecthyma gangrenosum associated with methicillin-resistant Staphylococcus aureus infection: A case report and review of the literature Cutis 2012;90:67–9.  Back to cited text no. 1
Sarkar S, Patra AK, Mondal M. Ecthyma gangrenosum in the periorbital region in a previously healthy immunocompetent woman without bacteremia. Indian Dermatol Online J 2016;7:36-9.  Back to cited text no. 2
[PUBMED]  [Full text]  
Bassetti M, Vena A, Croxatto A, Righi E, Guery B. How to manage Pseudomonas aeruginosa infections. Drugs Context 2018;7:212527.  Back to cited text no. 3
Korte AKM, Vos JM. Ecthyma gangrenosum. N Engl J Med 2017;377:e32.  Back to cited text no. 4
Özkaya Ö, Üsçetin I, Egemen O, Bingöl D, Akan M. Reconstructive procedure of lower lip defect due to ecthyma gangrenosum-A rare complication of acute lymphoblastic leukemia. J Craniofac Surg 2012;23:e182-84.  Back to cited text no. 5
Reich HL, Williams Fadeyi D, Naik NS, Honig PJ, Yan AC. Nonpseudomonal ecthyma gangrenosum J Am Acad Dermatol 2004;50 (5 Suppl):S114-7.  Back to cited text no. 6
Maccheron LJ, Groeneveld ER, Ohlrich SJ, Hilford DJ, Beckingsale PS. Orbital cellulitis, panophthalmitis, and ecthyma gangrenosum in an immunocompromised host with Pseudomonas septicemia. Am J Ophthalmol 2004;137:176-8.  Back to cited text no. 7
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 Global Infect Dis 2019;11:43-6.  Back to cited text no. 8
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