Ecthyma gangraenosum (EG) is an uncommon cutaneous infection classically associated with Pseudomonas aeruginosa, a non-fermentative, gram-negative bacillus bacteremia. The characteristic lesions of EG are hemorrhagic vesicles or pustules that evolve into necrotic ulcers with a tender erythematous border. EG was prescribed by Hitschmann and Kreibich after its first description by Barker in 1897 [1]. EG usually occurs in patients who are critically ill and immunocompromised. Although P. aeruginosa remains the most frequent organism identified in EG, other causative pathogens like other bacteria, including Proteus species, Escherichia coli, and methicillin-resistant Staphylococcus epidermidis, have been implicated in similar clinical presentations [2-5]. However, there are reports of the occurrence of EG in healthy individuals as well [5].

Here we are reporting a case of ecthyma gangraenosum which started as a small vesicular lesion in the face and later presented with extensive multiple similar-looking lesions in the trunk and extremities. All lesions undergone multiple stages of surgical debridement and vacuum drain along with culture-guided antibiotics therapy in a pediatric intensive care setup. Even though mortality in EG is high, aggressive meticulous management by a multidisciplinary team could revive the infant with minimal morbidity after an ICU stay of three weeks.

A 9-month-old male infant with an otherwise normal medical history and normal delivery presented to the Accident and Emergency Department of King Fahd Military Medical Complex Dhahran (KFMMCD) with complaints of a red blister and swelling on the right cheek, associated with fever and decreased oral intake [Figure 1]. The patient was initially given oral co-amoxiclave and paracetamol in the emergency department and was scheduled for a review in the Outpatient Department of Maxillofacial Surgery. Initially, the blister developed into red macules rapidly increasing in diameter, becoming glazy, violaceous-scaled, necrotic area surrounded by an erythematous halo [Figure 2]. The lesions subsequently ulcerated within the next 3-4 days. Similar lesions developed on the abdomen, right thigh, and left knee [Figures 3 and 4]. The skin lesions were accompanied by continuous fever and lethargy. There was no history of trauma or insect bites. The patient reported a history of travel to a countryside farm a week before this incident.

Figure 1: Early Presentation of Cheek Lesion Prior to Blister Formation.

Figure 2:Eschar With Central Bullae and Necrotic Spread With An Erythematous Halo.

Figure 3: Right Thigh Lesion with Hemorrhagic Center.

Figure 4: The Lesion in the Left Thigh and Back.6.

The patient was admitted on the second day and later shifted to the Pediatric Intensive Care Unit when showed signs shock. Upon examination, the baby was severely lethargic, hyperthermic (core temperature 101°F), tachypneic (respiratory rate 64/min), and refusing feeds. The lesions on the cheek, abdomen, and right thigh exhibited multiple, well-defined, punched-out gangrenous ulcers with raised erythematous borders and a central black eschar. The largest lesion, measuring 2 cm × 3 cm, was observed on the right cheek.

Investigations revealed a hemoglobin level of 12.6 g/dL, a total leukocyte count of 10,300/mm³, an absolute neutrophil count of 22,000/mm³, and platelets count of 159,000/mm³). The coagulation profile was deranged (international normalized ratio - 1.7). Chest radiography and TORCH profile were conducted. Initially, the parents refused cerebrospinal fluid examination, but it was performed two days later, and the results were normal. Lesions were swabbed for culture and sensitivity studies, and blood cultures were obtained. The blood culture grew Pseudomonas aeruginosa. Subsequent culture and Gram staining of the discharge from the skin, after 4 days, also showed the growth of Pseudomonas aeruginosa. The urine culture was sterile.

Based on the history suggestive of sepsis, typical skin lesions, and the growth of Pseudomonas aeruginosa in the blood culture, the diagnosis of ecthyma gangraenosum associated with Pseudomonas infection was made. Microscopic examination revealed an ulcerated zone of necrotic epidermis and dermis with abundant neutrophilic predominance. Early lesions with bullae showed basilar split mimicking a vesiculobullous lesion. [Figures 5 and 6].

Figure 5

Fig6: Showing Necrotic Area with Severe Neutrophil Infiltration.

Upon admission to our hospital, the child was empirically started on ceftriaxone and vancomycin. Based on the blood culture report, the antibiotics were changed to meropenem, amikacin, and vancomycin. On the third day of admission, the patient was taken to the operating theater. All wounds were debrided to remove necrotic tissue, and dressings were performed daily along with the application of local antibiotics (Fucidic acid), zinc oxide cream, and silver sulfadiazine. On the fifth day, the prolactin level was recorded as 9.05 mic/ml, and there was a continued reduction in albumin levels. Therefore, the patient was administered 10 mg of albumin along with total parenteral nutrition.

On day six, new lesions were noted on the left leg and just below the right knee. Further debridement was performed, and vacuum pump dressings were applied to the face, legs, and abdomen with a negative pressure of 50 mm Hg. Continuous monitoring of vancomycin levels helped in adjusting the dosage to an optimal level.

On day nine, the facial and abdominal lesions showed signs of healthy wound bed formation with a reduction in necrosis. However, the new lesions continued to expand with necrotic borders. On day eleven, new lesions were noted on the right hand and feet, but their size and aggressiveness were smaller compared to the previous lesions.

No new lesions were observed after two weeks of therapy. The vacuum dressing pressure was reduced to 40 mm Hg after 15 days, and the antibiotics were stopped after 19 days as all lesions showed signs of secondary epithelialization [Figures 7 and 8]. On day seventeen, negative results were obtained from a wound swab and blood culture. The patient underwent a full-thickness skin graft for the defect on the cheek, as the parents refused a vascular graft at this stage. The patient was discharged home after a total hospital stay of 4 weeks.

Figure 7: Healthy Granulation after Debridement.

Figure 8: The Patient Tolerates the Suction Drain on the Face.

The presented case report describes a 9-month-old male infant with ecthyma gangraenosum (EG) associated with a Pseudomonas aeruginosa infection. P. Aeruginosa is an opportunistic pathogen as it mostly causes infection in immunocompromised hosts such as premature infants [6]. In this case, an otherwise healthy infant is presented with characteristic EG lesions initially presented as a cheek blister progressed to multiple necrotic lesions all over the body.

An initial diagnosis of necrotizing fasciitis is ruled out by classic clinical presentation unique to ecthyma gangrenosum. In EG the lesions begin as erythematous macules in the skin, which advance to pus forming flat, usually round ulcers with surrounding redness and induration. Hemorrhagic areas appear at the center of the lesion, forming single or multiple bullae. Eventually, these bullae evolve into a gangrenous ulcer with a black eschar surrounded by a red halo with mild eversion of the edges [1-5]. These lesions may be single or multiple. This exact clinical picture is seen in this case.

P.Aeruginosa is primarily an environmental organism but highly capable of surviving in a wide range of conditions [1]. It is an opportunistic pathogen as it mostly causes infection in immunocompromised hosts such as premature infants [6]. With regards to bloodstream infection, it is predominantly associated with late-onset sepsis [7]. Bloodstream infection is suspected in this case as other modes of spread are ruled out.

The management of EG requires a multidisciplinary approach. Prompt initiation of appropriate antibiotic therapy is crucial, considering the potential for rapid disease progression and systemic spread. Empiric therapy with ceftriaxone and vancomycin was initiated upon admission, followed by adjustment based on the blood culture

Report. Meropenem, amikacin, and vancomycin were administered, targeting pseudomonas infection when confirmed with culture and sensitivity tests.

Surgical intervention played a significant role in this case. Debridement of necrotic tissue was performed four times within two weeks time to establish a healthy wound bed. Daily dressings, including the application of local antibiotics and wound care products, were done to facilitate healing. The use of vacuum pump dressings with negative pressure helped promote wound healing and prevent infection.

Throughout the treatment course, close monitoring of the patient's condition and laboratory parameters was essential. Serial measurements of prolactin, albumin levels, and vancomycin levels aided in assessing the patient's response to treatment and guiding therapeutic adjustments.

The progression of the skin lesions, in this case, demonstrated both positive and negative aspects. While the facial and abdominal lesions showed signs of healthy wound bed formation with a reduction in necrosis, new lesions continued to appear on other body regions, albeit with smaller sizes and less aggressiveness. The overall response to treatment was seen as favorable only after two weeks as no new lesions were observed after this. Eventually, all existing lesions exhibited signs of secondary epithelialization.

P.aeruginosa sepsis was associated with high mortality (50%) and morbidity (83.3%).(Ref). The parent’s decision to defer early biopsy for diagnostic confirmation, as well as the refusal of early debridement, introduced certain limitations to this case. However, characteristic skin lesions, positive blood culture for P. aeruginosa, response to targeted antibiotic therapy, multiple surgical debridement's, and multidisciplinary team management collectively supported the successful revival of the infant.

This case report highlights the importance of considering EG as a potential diagnosis in patients presenting with characteristic skin lesions and systemic manifestations, particularly in the context of a Pseudomonas infection. Early recognition, appropriate antibiotic therapy, and surgical intervention are crucial for successful management. Further research and case reports are warranted to enhance our understanding of this rare condition and optimize treatment strategies.

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal relatives have given their consent for images and other clinical information to be reported in the journal. The relatives understand that patient’s name and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.

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Conflicts of Interest

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