Ecthyma gangrenosum in the periorbital region in a previously healthy immunocompetent woman without bacteremia.

Ecthyma gangrenosum (EG) is a cutaneous lesion classically associated with potentially fatal Pseudomonas septicemia in immunocompromised patients. Other bacterial and fungal pathogens have also been implicated. Although EG typically occurs in immunocompromised or neutropenic patients, it may occasionally affect a previously healthy person. The cutaneous findings are characteristic with small indurated papulovesicles progressing rapidly to necrotic ulcers with surrounding erythema and a central black Eschar. While lesions can occur at any site, most are commonly found over the buttocks, perineum, limbs, and axillae. We describe a case of EG in periorbital region in a previously healthy woman who responded to appropriate antibiotic treatment for Pseudomonas. It is very important to establish the diagnosis early so that appropriate systemic antibiotic therapy can be initiated to reduce morbidity and potential mortality.

INTRODUCTION

Ecthyma gangrenosum (EG) is a characteristic vesicular and necrotic skin manifestation of systemic sepsis commonly caused by Pseudomonas aeruginosa. It was first described in association with Pseudomonas septicemia by Barker in 1897 and was later given the name “ecthyma gangrenosum” by Hitschmann and Kreibich.[1] It is usually seen in critically ill patients with neutropenia or underlying immune deficiency who present with skin lesions that begin as an erythematous, nodular or hemorrhagic vesicles that turn into necrotic ulcers with eschar formation.[2] The skin lesions are usually widespread over the area involved. It initially appears as a painless round erythematous macule, which rapidly turns into a pustule with surrounding erythema. A hemorrhagic bulla appears in the center of the lesion, which spreads peripherally evolving into a gangrenous ulcer with a central black or gray eschar surrounded by a characteristic erythematous halo. Patients often present with fever a few days prior to developing EG. Children with EG may develop diarrhea (30%) before onset of cutaneous lesions. EG is usually seen in immunocompromised patients with leukemia, lymphoma, other malignant diseases, severe burns, organ transplants, or in people receiving immunosuppressive therapy. However a few case reports describe the development of EG in previously healthy patients without any of the unrecognized risk factors such as intraabdominal abscess, recent viral illness, antimicrobial treatment for neutropenia or hypogammaglobulinemia. EG occurs in 1.3%–3% patients with P. aeruginosa sepsis. It had been considered to be pathognomonic of Pseudomonas sepsis until it was described in cases with infections caused by Group A Streptococcus, Aeromonas hydrophila, Escherichia coli, Candida albicans, Aspergillus species, and Mucor species.[34] The mechanism of action of tissue destruction by Pseudomonas is due to exotoxin A, which inhibits protein synthesis; Elastase, which degrades elastin in blood vessel wall; Phospholipase C, which degrades phospholipids in cell membrane and pyocyanin that generates reactive oxygen species.[3] EG may appear at any location on the body but predominantly affects anogenital and axillary areas. Frequency of distributions - gluteal/perineal (57%), extremities (30%), trunk (6%), and face (6%). Bilateral periorbital manifestations are rare but have been reported.[4] Here our case of EG is over the periorbital region in an immunocompetent healthy woman without bacteremia.

CASE REPORT

A previously healthy 35-year-old woman was admitted with a 2 weeks history of a crusted ulcer around left eye. The lesion started at outer canthus of the left eye as a painless erythematous pustular lesion with surrounding edema, which later spread in the next 7–8 days to involve upper and lower eyelids along with swelling of the cheek on the left side. There were multiple hemorrhagic bullae that ruptured to form a gangrenous ulcer with an overlying eschar [Figure 1].

Figure 1

Crusted and gangrenous plaque around left eye

Patient complained of watering from her left eye along with gradual diminution of vision as a result of orbital swelling obscuring the vision. There was no history of fever before or after the appearance of the lesion.

The patient did not give any past history of diabetes mellitus or chronic respiratory illness or any abnormal bleeding. There was no significant drug history, or history of insect bite, or intimate contact with cattle.

General examination showed no pallor, icterus, cyanosis, clubbing, or pedal edema. The patient was of average build and height with no signs of malnutrition. Pulse, blood pressure, and temperature were within normal limits. No abnormality was detected in the respiratory and cardiovascular systems. Abdomen was soft with normal bowel sounds. The liver, spleen, and lymph nodes were not palpable. Central nervous system examination was within normal limits. The patient was well oriented to time, place, and person.

Local examination showed a crusted ulcer situated at the left periorbital region of size 9 cm × 5 cm with irregular shape, ragged margins, raised hyperpigmented border, and floor filled with slough and necrotic tissue. The lesion was nontender and the base was indurated.

Blood investigations such as complete hemogram, blood sugar, serum urea and creatinine, liver function test, and serum electrolytes were within normal limits. Routine and microscopic examination of urine showed no abnormality. C-reactive protein was within normal range. Serology for human immunodeficiency virus -1 and 2 was nonreactive. Chest radiograph posteroanterior view and ultrasound whole abdomen were normal. Blood culture showed no growth of microorganism. Swab taken from the ulcer showed growth of P. aeruginosa in both blood agar and MacConkey agar media. Punch skin biopsy from the margin of the ulcer stained with hematoxylin and eosin showed necrosis of the epidermis and the upper dermis, covered on the surface by necrotic exudates [Figure 2]. The deeper dermis and subcutaneous fibroadipose tissue also showed necrotic areas surrounded by inflammatory cells with a predominance of polymorphonuclear neutrophils. The epidermis adjacent to the necrotic areas showed hyperkeratosis and acanthosis. No vasculitis was found [Figure 3].

Figure 2

H and E ×10: Necrosis of the epidermis with necrotic exudate in the dermis

Figure 3

H and E ×40: Necrotic fibroadipose tissue with predominance of polymorphonuclear neutrophils

Systemic antipseudomonan antibiotic with injection piperacillin and tazobactam 4.5 g thrice daily was started after obtaining the wound swab culture sensitivity report. The lesion completely resolved with subsidence of orbital swelling within 14 days of antibiotic therapy. The patient regained vision in her left eye completely at the end of treatment [Figure 4].

Figure 4

Healed lesion after antipseudomonal antibiotic

Considering the clinical findings, laboratory investigations and therapeutic response to antipseudomonas antibiotics the case was diagnosed as ecthyma gangrenosum without bacteremia.

DISCUSSION

It is well known that EG is a rare cutaneous lesion of systemic infection caused most commonly by P. aeruginosa. It is characterized by round or oval lesions with erythematous halo and a necrotic center representing where the organism invades blood vessels and causes infarctions. It is observed commonly in patients with any kind of immunodeficiency.

Although the commonest site of EG lesions is the gluteal or perineal region (57%), a few atypical sites may be involved such as extremities, trunk, face, and rarely periorbital region.[25] In our case, EG occurred in periorbital region, which is uncommon.

Majority of EG were reported in patients with septicemia and immunocompromised status such as leukemia, lymphoma, other malignant diseases, severe burns, organ transplant, or in people receiving immunosuppressive therapy or recent antibiotic therapy for neutropenia or hypogammaglobulinemia. In our case we could not find any systemic illness or other factors mentioned above.[23467]

There are a few case reports that EG can present as localized skin eruptions that are not accompanied by bacteremia or systemic infection. The source of infection in this patient could not be determined with certainty, but it is possible that the patient presented with erysipelas, which was later colonized and superinfected with P. aeruginosa and then developed EG. Negative blood culture suggests that EG developed as a primary lesion at the mentioned site.[2345]

The commonest causative organism of EG is P. aeruginosa, but other organism such as Group A Streptococcus, Staphylococcus aureus, Escherichia coli, Aeromonus hydrophila, Serratia marcescens, and so on can also cause EG. In our case although bacteremia was not detected but the wound swab showed growth of pseudomonas.[8]

Although usual outcome is poor, rapid diagnosis, and aggressive appropriate systemic treatment of EG can lead to successful outcome. Effective intravenous antipseudomonas therapy (beta-lactam antibiotic with or without an aminoglycoside) is appropriate for treatment of both bacteremic and nonbacteremic EG. Absence of bacteremia is associated with the best outcome. Patients with Pseudomonas bacteremia have been reported to have a mortality rate of 38%. On the other hand only two patients (15%) died in a review of 13 patients with EG without bacteremia.[9] In another study, the mortality rate was 7.5% in the group of patients with skin lesions considered to be primary and 20% in the group of patients with skin lesions considered to be secondary to bacteremia.[10] Factors that are associated with higher mortality include neutropenia, septic shock, inappropriate or delayed antibiotic therapy, and resistant microorganisms. Our case is unique in the sense that it occurred in an immunocompetent healthy female, not associated with bacteremia and the site was periorbital region, which is not a common site of the disease described in the literature.

Declaration of patient consent

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Conflicts of interest

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