Ecthyma gangraenosum L08.4

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 14.06.2022

Synonym(s)

Dermatitis gangraenosa infantum; ecthyma cachectoricum; Ecthyma gangraenosum terebrans; ecthyma terebrans; Gangraena multiplex cachecticorum; varicella gangraenosa

Definition
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Septic, highly febrile clinical picture with painful, initially furunculoid, then torpidly decaying, abscessing, highly red infiltrates, usually due to intercurrent Pseudomonas sepsis. Patients with acquired or congenital (see primary immunodeficiencies) immunodeficiencies are affected.

Pathogen
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In a larger cross-sectional study of 167 cases, P. aeruginosa was detected in 123 cases (73.65%), septicemia in 72 patients (58.5%). Another bacterial etiology(E.coli, Proteus) was detected in 29 cases (17.35 %). Fungi (?) detected in 15 cases (9 %) (Vaiman M et al. 2015)....

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Etiopathogenesis
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The generally accepted definition of ecthyma gangrenosum (EG) is that this condition is pathognomonic for Pseudomonas septicemia (Pseudomonas aeruginosa) and usually occurs in immunocompromised patients.

Note: The vast majority of publishers make the diagnosis of "ecthyma gangraenosum" only when there is evidence of Pseudomonas infection.

Manifestation
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Especially in immunodeficient infants or young children; also in long-term immunocompromised (e.g., organ transplanted) adults or debilitated elderly.

Localization
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The disease mainly affects the armpits and the anogenital region; arms, legs, trunk and face are less frequently affected. A distinction must be made here from chancriform (staphylogenic) pyoderma.

Clinical features
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Life-threatening, septic clinical picture with focal edema, central hemorrhagic pustule formation, soft, small-nodular, initially furunculoid elevations . Within hours, torpid decay with formation of large-arched, extensive ulcerations with elevated, hemorrhagic margins.

Diagnosis
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Antibiogram and pathogen detection from swabs.

Differential diagnosis
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Furuncle (no septic clinical picture, mostly staphylococci, intial similar course, but no phlegmonous spread).

Chancroid pyoderma: predilection=face; causative agent usually staphylococci;

Botryomycosis: chronic course with sporotrichoid nodules; usually Staphlococcus aureus;

External therapy
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Removal of necrotic and bacteria-containing coatings, wound dressings with polyvidon iodine ointments(e.g. Braunovidon iodine ointment).

Internal therapy
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Resistance to antibiotics is to be expected. Therefore treatment after an antibiotic biogram. In case of severe infections always combination therapy, e.g. with an acylureidopenicillin (e.g. piperacillin). Alternatively, ceftriaxone (e.g. Rocephin) once/day 2 g i.v. Alternatively, ciprofloxacin (Ciprobay) combined with an aminoglycoside such as tobramycin (Gernebcin) Start therapy as soon as possible, in case of suspicion even before the antibiogram.

Operative therapie
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Consistent renovation of entry ports.

Progression/forecast
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Serious, danger of sepsis.

Literature
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Barasch A et al (2003) Necrotizing stomatitis: report of 3 Pseudomonas aeruginosa-positive patients. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 96: 136-140.
Fukui KO et al (2019) Ecthyma gangrenosum in a 3-year-old boy post-heart transplant. Transpl Infect Dis 21:e13131.
Gałązka P et al. (2021) Ecthyma gangrenosum as a serious complication of Pseudomonas aeruginosa infection in departments of paediatric oncology. Postepy Dermatol Allergol 38:537-543.
Gkoufa A et al (2022) Challenging cutaneous lesion in a patient With Chronic Idiopathic Neutropenia. Cureus 14:e21225.
Ishihara T et al (2021) Ecthyma gangrenosum mimicking cellulitis. Clin Case Rep 9: 1787-1788.
Kelley DJ (2003) Ecthyma gangrenosum of the nasal cavity. Otolaryngol Head Neck Surg 129: 754-755.
Levy I et al (2003) Ecthyma gangrenosum caused by disseminated Exserohilum in a child with leukemia: a case report and review of the literature. Pediatr Dermatol 20: 495-497
Maccheron LJ et al (2004) Orbital cellulitis, panophthalmitis, and ecthyma gangrenosum in an immunocompromised host with pseudomonas septicemia. Am J Ophthalmol 137: 176-178.
Pulido J et al (2021) Labial ecthyma gangrenosum in an immunocompromised infant with leukemia: heightening awareness for the urologist. Urology 80:1366-1368.
Shah M et al (2022) Ecthyma gangrenosum. 2022 May 8. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. PMID: 30521198.
Vaiman M et al. (2015) Ecthyma gangrenosum and ecthyma-like lesions: review article. Eur J Clin Microbiol Infect Dis 34:633-639.
Watson A, Sloan B (2003) Ecthyma gangrenosum arising from Pseudomonas aeruginosa dacryocystitis. Clin Experiment Ophthalmol 31: 366-368.