Ecthyma Gangrenosum: A Life-threatening Complication of Diaper Rash in a Healthy Infant

To the Editors: Ecthyma gangrenosum (EG) is a deep cutaneous infection mostly caused by Pseudomonas aeruginosa.1 The mortality is high, reaching 50%–77% in bacteremic cases and 15% for localized forms,1,2 which may progress to secondary bacteremia if inadequately treated.3 While typically affecting the immunocompromised,4 it can occur in immunocompetent individuals with prolonged skin barrier disruption, as in severe diaper rash.1,5 Few cases of diaper rash-related EG are described, and even fewer describe it in immunocompetent patients. A male toddler presented with prostration, high fever, diarrhea, severely worsening diaper rash and rapidly evolving gluteal lesions. He had intermittent fever for a month and had presented repeatedly to another emergency department with cough and respiratory distress, having completed antibiotic therapy 17 days before presentation. He was irritated, febrile and tachycardic, with necrotizing perianal lesions (Fig. 1A). The mother’s photographs enlightened the rapid aggravation in few hours.FIGURE 1.: Evolution of the lesions. A: at presentation in the emergency department, necrotic lesions with an inflammatory halo can be seen. B: On day 4 after admission, after the initial debridement and negative-pressure dressing. C: On day 6 after admission, before the colostomy. D: 20 days after admission, after the debridement and before the dermal substitute. E: after the dermal substitute. F: day 7 after the dermal substitute. G: on day 21 after the dermal substitute. H: minor hypertrophy of the scar 2 months after hospital discharge.He had markedly elevated C-reactive protein (442 mg/L) and mild leukopenia (4.02 × 109/L). Neutropenia (1.35 × 109/L) was only observed at admission, with no recurrence during hospitalization. Blood cultures isolated Pseudomonas aeruginosa, and wound swabs Pseudomonas aeruginosa, Proteus mirabilis and Enterococcus faecalis. With concerns about sepsis, the child was submitted to emergent surgical debridement of the lesions and negative-pressure dressing. He was admitted to intensive care with empiric antibiotics (metronidazole, vancomycin and piperacillin-tazobactam) (Fig. 1B). A loop colostomy was created 7 days after admission due to wound stool contamination, with anal sphincter exposure (Fig. 1C). Twenty days after admission, after multiple debridements and negative-pressure therapy dressings, lesions were further aggravating, so the dermis was substituted by a dermal regenerative matrix (NovoSorb biodegradable temporizing matrix—PolyNovo, Australia), accompanied by a negative-pressure dressing (Fig. 1D–F). This was followed by a partial-thickness skin graft to substitute the epidermis, performed on the 21st day after the placement of the dermal matrix (Fig. 1G). The graft was collected from the inner thigh with placement of an epidermal substitute (SUPRATHEL—Polymedics, Germany) in the donor area. There was minor partial graft loss, with full recovery 1 week after this last procedure. The colostomy was closed 4 months after discharge. The child was investigated for immunodeficiency, but there was no pathological finding or abnormality suggesting immunocompromise: no further neutropenia was observed during hospitalization or follow-up; immunoglobulin levels, immunophenotyping and oxidative burst tests were normal. A next-generation sequencing panel for primary immunodeficiency disorders and screening for secondary causes was also negative. Nine months after discharge, the child is thriving with a normal diet and transit; there was only minor hypertrophy of the scar (Fig. 1H). This report adds to the literature by documenting a diaper-rash-associated perineal EG in a healthy child, a rare condition for which awareness is essential; the urge for emergent medical and surgical treatment must not be overlooked. In perianal infections requiring colostomy, this procedure must not be delayed as fecal contamination further worsens the lesions, precluding healing. Synthetic dermal matrices and skin grafts are feasible in deep cutaneous necrosis, with good functional and esthetic outcomes.