Presentation
This patient’s presentation was unusual for the diffuse presentation of non-bacteremic EG with panniculitis. [scholarlycommons.hcahealthcare.com]
Congenital agammaglobulinemia presenting with ecthyma gangrenosum. Acta paediat. scand., 80: 732-734, 1991. 11. [scielo.br]
Methods A one-and-a-half-year-old girl presented with a history of recurrent (respiratory) infections and the sporadic occurrence of purpuric, vulvar ulcers. [onlinelibrary.wiley.com]
As the present case demonstrates, this critical complication has the potential to arise after routine operations, including elective neurosurgical procedures. [cureus.com]
We wanted to present this important loss of function occurring secondary to ecthyma gangrenosum in light of present available literature. 2. Case Report A male infant was vaginally delivered to a 24-year-old G2P2 mother at 30 weeks of gestation. [hindawi.com]
Entire Body System
- Candidiasis
The most common Candida (C) species to result in candidiasis is C. albicans. [dermnetnz.org]
File TM Jr, Marina OA, Flowers FP (1979) Necrotic skin lesions associated with disseminated candidiasis. Arch Dermatol 115(2):214–215 Article PubMed Google Scholar 27. [link.springer.com]
- Pathologist
EG was first described in 1897 by Canadian pathologist Dr. [statpearls.com]
- Fulminant Sepsis
For individuals with fulminant sepsis, management in the critical care setting by an intensivist is appropriate due to the high fatality rate of bacteremic EG. [statpearls.com]
Respiratoric
- Productive Cough
For example, productive cough or dysuria may reveal a respiratory or urinary source, respectively. In the non-bacteremic form, it is important to note any trauma that may have introduced the infection via direct inoculation. [statpearls.com]
Cardiovascular
- Hypertension
Case Report: A 62-year-old man with hypertension and hyperlipidemia presented to the emergency department for pain, swelling, and blackish discoloration of the right lower extremity. He suffered a crush injury to the right great toe one week prior. [journalofscientificinnovationinmedicine.org]
This case involves a 63-year-old female patient, with hypertension, type 2 diabetes and a kidney transplant from a cadaver donor 3 years earlier; she attended the emergency room for a two day history of generalised skin lesions, fever and sore throat. [revistanefrologia.com]
Her previous medical history includes essential hypertension, diabetes mellitus and breast cancer, which was diagnosed four months ago and is currently under neoadjuvant chemotherapy (last treatment was two weeks before admission). [journals.lww.com]
The American journal of case reports. 2014 Oct 31 [PubMed PMID: 25360696] [18] Hafner J, Calciphylaxis and Martorell Hypertensive Ischemic Leg Ulcer: Same Pattern - One Pathophysiology. [statpearls.com]
Skin
- Skin Lesion
EG can be confused with other skin lesions in newborns like noma neonatorum and the skin lesions associated with infections such as group A streptococcus, Aeromonas hydrophila, Staphylococcus aureus, Serratia marcescens, Pseudomonas maltophilia, Escherichia [hindawi.com]
The main mechanism of the organism that is causing the typical skin lesions is the invasion of the organism into the arteries and veins in the dermis and subcutaneous tissues of the skin. [en.wikipedia.org]
On day 3, Pa was identified in the blood and the skin lesion cultures, and the antibiotic regimen was immediately changed to 200 mg/kg/day ceftazidime IV. The skin lesions became more necrotic and spread further. [journals.lww.com]
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 [3]. [jmedicalcasereports.biomedcentral.com]
Similar lesions developed on the back, gluteal region, and both lower limbs. The skin lesions were accompanied by a continuous low-grade fever. There was no history of burns, drug intake, umbilical sepsis, catheterization, or diarrhea. [escholarship.org]
- Cutaneous Manifestation
Physicians should become aware of the cutaneous manifestations of underlying Pseudomonas infection so appropriate antibiotic therapy can be initiated. [scholarlycommons.hcahealthcare.com]
Ecthyma gangrenosum: a rare cutaneous manifestation of a potentially fatal disease. Ann Otol Rhinol Laryngol 2004;113:462–4. [4]. Puzenat E, Chirouze C, Khayat N, et al. [journals.lww.com]
Ecthyma gangrenosum: A rare cutaneous manifestation caused by pseudomonas aeruginosa without bacteraemia in a leukaemic patient- A case report. Indian J Med Microbiol 2005;23:262-263. PubMed [PubMed - indexed for MEDLINE] 4. Kao J, Low D.E, Gan SB. [escholarship.org]
Ecthyma gangrenosum: a rare cutaneous manifestation caused by Pseudomonas aeruginosa without bacteremia in a leukemic patient. Indian J Dermatol Venereol Leprol 2005;71:128-9. Tsao H, Swartz M, Weinberg A, Jonson RA. [medigraphic.com]
Ecthyma gangrenosum: A rare cutaneous manifestation caused by Pseudomonas aeruginosa without bacteremia in a leukemic patient. Indian J Dermatol Venereol Leprol 2005;71:128-9. [PUBMED] 6. Wolf JE, Liu HH, Rabinowitz LG. [idoj.in]
- Hyperkeratosis
The epidermis adjacent to the necrotic areas showed hyperkeratosis and acanthosis. No vasculitis was found [Figure 3]. [idoj.in]
Microscopic examination reveals an ulcerated zone of necrotic epidermis and dermis with neutrophilic predominance.[14][15] The epidermis adjacent to the necrotic region reveals reactive hyperkeratosis. [statpearls.com]
- Furunculosis
Ecthyma gangrenosum is usually seen in immunocompromised patients and in healthy patients with a history of foliculitis and furunculosis, and use of swimming pool and jacuzzi. [medigraphic.com]
- Necrolytic Migratory Erythema
Other conditions with similar macroscopic features include autoimmune vasculitides, vasopressor-induced necrosis, calciphylaxis, warfarin-induced necrosis, and disseminated intravascular coagulation (DIC), pyoderma gangrenosum, necrolytic migratory erythema [statpearls.com]
Urogenital
- Dysuria
For example, productive cough or dysuria may reveal a respiratory or urinary source, respectively. In the non-bacteremic form, it is important to note any trauma that may have introduced the infection via direct inoculation. [statpearls.com]
Workup
Objectives We report the workup of a case of vulvar EG caused by P. aeruginosa in a toddler, which led to a diagnosis of an underlying neutropenia. [onlinelibrary.wiley.com]
During the second PICU day, the infant underwent surgical debridement of the necrotic lesion (Fig. 3), and due to the severe evolution and marked neutropenia, a complete workup for immunodeficiency was made in association with the empirical use of human [scielo.br]
A dermatologist can help in the initial diagnostic workup to determine the cause (Wood's lamp evaluation, punch biopsy) and extent of the lesions. [statpearls.com]
Serum
- Neutropenia
Treatment with G-CSF could eventually be stopped, and the neutropenia, ultimately transient in nature, completely resolved. [onlinelibrary.wiley.com]
Ecthyma gangrenosum is an uncommon cutaneous infection usually associated with Pseudomonas aeruginosa bacteraemia, and occurs invariably in unwell and immunocompromised patients, especially when there is a prolonged spell of neutropenia. [pcds.org.uk]
Neutropenia and malignancy in a patient with typical skin lesions should raise suspicion for EG. [statpearls.com]
Patient 1: Male, 36 years old, with acute lymphoblastic leukemia, presented with febrile neutropenia (FN) during chemotherapy, and treatment with cefepime was started. [anaisdedermatologia.org.br]
On the second day of life neutropenia was detected, requiring treatment with a colony-stimulating factor. [actasdermo.org]
- Leukopenia
In a study reported by Huang et al, leukopenia was present in 24 (57%) of 43 children evaluated. [journals.lww.com]
[…] with ecthyma gangrenosum, with neutropenia being one of the main factors for poor prognosis.8 In a series of 43 cases of immunocompetent paediatric patients who developed sepsis due to P. aeruginosa, 9 out of 10 patients who died had presented with leukopenia [revistanefrologia.com]
The presence of neutropenia and leukopenia are a poor prognostic sign. Skin biopsy will show a necrotizing vasculitis, necrosis of the epidermis, or complete ulceration. Multiple bacteria may be seen in the vessel lumen and within the dermis. [dermatologyadvisor.com]
Microbiology
- Pseudomonas
Ecthyma gangrenosum; Pseudomonas aeruginosa; Agammaglobulinemia; Infant Éctima Gangrenosa (EG) por Pseudomonas aeruginosa é uma infecção rara e invasiva que pode ser associada com agamaglobulinemia. [scielo.br]
An MRI revealed myositis and edema in the right paraspinal region, and blood cultures were negative for Pseudomonas. [scholarlycommons.hcahealthcare.com]
Cultures of blood, pus, and biopsy grew Pseudomonas aeruginosa. Skin biopsy revealed pauci-immune necrotizing vasculitis with hemorrhage and surrounding edema. The diagnosis of ecthyma gangrenosum associated with Pseudomonas infection was made. [escholarship.org]
Blood culture revealed growth of Pseudomonas aeruginosa, sensitive only to imipenem. The infant died the day following admission. References 1 Ishikawa, T., Sakurai, Y., Tanaka, M. et al.. [magonlinelibrary.com]
It is classically associated with Pseudomonas aeruginosa bacteremia, but it is not pathognomonic.[1] Pseudomonas aeruginosa is a gram negative, aerobic bacillus.[2] This type of skin lesion was first described in association with Pseudomonas aeruginosa [en.wikipedia.org]
- Candida
Candida skin problems See more images of candida infection. [dermnetnz.org]
Two weeks later, a control ulcer culture showed Candida albicans and Proteus sp. Then, fluconazol and cefuroxim were added for 10 days, until the ulcers disappeared. [medigraphic.com]
A lesion resembling EG but caused by Candida albicans was reported in a 12-day-old baby [10]. Another rare manifestation of pseudomonas infection in the neonate is noma neonatorum [11]. [hindawi.com]
It is most commonly caused by P. aeruginosa, however, it can also be caused by other bacteria (Aeromonas, Escherichia coli, Haemophilus influenzae) as well fungi (Candida, Fusarium, Zygomycetes).3 Prompt diagnosis and management with empirical antimicrobials [casereports.bmj.com]
Moreover, some fungi, such as species of Candida, Aspergillus and Curvularia, have been reported to cause clinically similar lesions.2 Some authors define the disease according to its aetiological agent, while others define it by its clinical characteristics [elsevier.es]
- Corynebacterium Diphtheriae
The mechanism of tissue destruction is Pseudomonas exotoxin A, a toxin similar to Corynebacterium diphtheriae toxin that causes inactivation of elongation factor 2 thus inhibition of protein synthesis, elastase, an enzyme which degrades elastin in blood [wikidoc.org]
diphtheriae, Klebsiella pneumoniae, Neisseria More Details gonorrhoeae, Staphylococcus aureus, Streptococcus pyogenes, and Yersinia More Details pestis, and fungi such as Aspergillus fumigatus, Candida albicans, and herpes simplex virus.[3] Initially [ijpd.in]
diphtheriae Escherichia coli Klebsiella pneumoniae Morganella morganii [14] Neisseria gonorrhea Pseudomonas aeruginosa Pseudomonas stutzeri Serratia marcescens Vibrio vulnificus Yersinia pestis Xanthomonas maltophilia Fungi are as follows: Aspergillus [emedicine.medscape.com]
diphtheriae, Neisseria gonorrhea, Yersinia pestis, fungi like the Candida species), and viruses like herpes simplex virus.[1][5][6][7] Epidemiology Ecthyma gangrenosum affects all age groups and genders. [statpearls.com]
Treatment
On the second day of life neutropenia was detected, requiring treatment with a colony-stimulating factor. [actasdermo.org]
Five days after the treatment initiation the skin lesions started to improve and on the 10th day of treatment these lesions disappeared; however, due to the loss of tissue in the lips, soft palate, and tongue, the patient developed difficulty in the functions [hindawi.com]
The patient did not survive depicting of the treatment. Patient 2: Male, 47 years old, had FN during chemotherapy for acute myeloid leukemia, and treatment with cefepime was started. [anaisdedermatologia.org.br]
Attempted treatments included broad-spectrum antibiotics, milrinone, vasopressors, and prone positioning, but the patient expired following cardiac arrest attributable to respiratory acidosis and hypoxia. [cureus.com]
Prognosis
Discussion Ecthyma gangrenosum usually responds well to systemic i.v. antipseudomonas treatement.[4],[5] But prognosis depends upon the underlying condition. [ijdvl.com]
Patients with this particular subtype of infection appear to have a better prognosis than those having a preceding bacteremia. (Arch Intern Med 1987;147:299-301) Full Text [jamanetwork.com]
The prognosis for children with Pa infection is influenced by several risk factors.[16] The first of these is the presence of an unknown immune deficiency. [journals.lww.com]
An early diagnosis and antibiotic treatment are essential to improve the prognosis of the disease. [revistanefrologia.com]
Etiology
We report a rare etiology of EG in an immunocompetent patient caused by methicillin-sensitive Staphylococcus aureus (MSSA). [journalofscientificinnovationinmedicine.org]
Freeman et al.10 later included these cases of noma neonatorum in the description of EG because of their common etiologic pathogen, P aeruginosa, which is practically inexistent in classic noma. [actasdermo.org]
Etiology P. aeruginosa is the most frequent organism identified in ecthyma gangrenosum, with one study identifying P. aeruginosa in 74% of cases.[1] P. aeruginosa is a non-lactose fermenting gram-negative aerobic, rod-shaped bacteria. [statpearls.com]
Etiology The ubiquitous gram-negative facultative anaerobic bacteria P aeruginosa is the causative bacteria. The bacteria are found routinely in soil and water sources. [dermatologyadvisor.com]
Epidemiology
Epidemiology and outcome of Pseudomonas aeruginosa bacteremia, with special emphasis on the influence of antibiotic treatment. Analysis of 189 episodes. Arch Intern Med. 1996; 156: 18, 2121–2126. [magonlinelibrary.com]
[…] are as follows: Aspergillus fumigatus Candida albicans [15] Curvularia species [16] Exserohilum species Fusarium solani [17] Mucor and Rhizopus species Pseudallescheria boydii [16] Scytalidium dimidiatum Viral causes include herpes simplex virus. [18] Epidemiology [emedicine.medscape.com]
[…] hydrophila, Klebsiella pneumoniae, Serratia marcescens, Xanthomonas maltophilia, Morganella morganii, Corynebacterium diphtheriae, Neisseria gonorrhea, Yersinia pestis, fungi like the Candida species), and viruses like herpes simplex virus.[1][5][6][7] Epidemiology [statpearls.com]
Pathophysiology
Pathophysiology The lesion is caused by perivascular invasion of bacteria in the dermis and subcutaneous tissue. [wikidoc.org]
The American journal of case reports. 2014 Oct 31 [PubMed PMID: 25360696] [18] Hafner J, Calciphylaxis and Martorell Hypertensive Ischemic Leg Ulcer: Same Pattern - One Pathophysiology. [statpearls.com]
Pathophysiology Impaired humoral or cellular immunity leads to increased susceptibility to infections with P aeruginosa or other pathogens. [emedicine.medscape.com]
Pathophysiology Small cutaneous blood vessels (venules and capillaries) become congested with bacteria. The bacteria activate the complement cascade and an inflammatory nidus is formed. Subsequent vasculitis and necrosis at the local site occurs. [dermatologyadvisor.com]
Prevention
[…] skin biopsies are required, one for histology (light microscopy), and a second for tissue culture looking for bacteria, fungi, and mycobacteria Management Ecthyma General measures In the short-term patients should use separate towels and flannels to prevent [pcds.org.uk]
Prevention measures include practicing proper hygiene, educating the immunocompromised patients for awareness to avoid possible conditions and seek timely medical treatment.[4] Treatments[edit] Treatments involve antibiotics that cover for Pseudomonas [en.wikipedia.org]
Use a high-efficiency particulate air filter (HEPA) in hospital to prevent hospital-acquired lung infection in patients with poor immunity. [dermnetnz.org]
The bronchoscopy showed thickening of the left bronchial tree and erythaema of the mucosa at the entrance to the lung base that prevented the passage of the bronchoscope. Bronchoalveolar lavage (BAL) was performed, finding no malignant cells. [elsevier.es]