Multidisciplinary management of disseminated Exophiala dermatitidis mycosis in an infant with mixed phenotype acute leukemia: a case report.

BACKGROUND: Exophiala dermatitidis is a dematiaceous fungus isolated from various environmental sources. Systemic E. dermatitidis infections can lead to fatal outcomes, and treatment has not yet been standardized. Although E. dermatitidis is also known to cause cutaneous infection, it has not been previously reported to appear as ecthyma gangrenosum (EG), an uncommon cutaneous lesion in neutropenic patients that is mainly caused by Pseudomonas aeruginosa. CASE PRESENTATION: A 2-month-old male infant with mixed-phenotype acute leukemia presented with prolonged fever unresponsive to antibacterial and antifungal agents during myelosuppression due to remission induction therapy. He also presented with skin lesions on the left wrist and left lower quadrant of the abdomen. The abdominal lesion gradually turned black and necrotic, which was consistent with the findings of the EG. E. dermatitidis was isolated from the blood, stool, wrist skin, and endotracheal aspirate. During hematopoietic recovery, consolidation in both lungs was evident. Multiagent antifungal treatment failed to eliminate E. dermatitidis from blood. In order to salvage the central venous catheter, ethanol lock therapy (ELT) was adopted, following which the blood culture became negative. The abdominal lesion that evolved as a necrotic mass connecting the small intestine and subcutaneous tissue adjacent to the skin was surgically resected. After these interventions, the general condition improved. CONCLUSION: Disseminated E. dermatitidis mycosis in the neutropenic infant was successfully managed with a multidisciplinary treatment consisting of multiagent antifungal treatment, ELT, and surgery.

Ecthyma gangrenosum caused by Staphylococcus aureus in hematological malignancies: Case reports and literature review.

RATIONALE: Ecthyma gangrenosum (EG) is a potentially life-threatening, systemic infection generally caused by Pseudomonas aeruginosa. Data on EG caused by Staphylococcus aureus in patients with hematological malignancies are scarce. The present case report aimed to describe the clinical features of EG caused by S. aureus in patients with hematological malignancies and to provide a comprehensive review of previous studies on the topic. PATIENT CONCERNS: The first patient was a 61-year-old man with acute myeloid leukemia who presented fever and multiple lesions during chemotherapy. The second patient was a 47-year-old man with myelodysplastic syndrome who developed progressive erythematous necrotic plaques on his extremities and face. DIAGNOSIS: Both cases were diagnosed as EG caused by S. aureus. While the first patient had concurrent methicillin-resistant S. aureus (MRSA) bacteremia, the second patient had positive results only for tissue culture of the skin lesion isolated methicillin-sensitive S. aureus. INTERVENTIONS: Vancomycin was initiated with critical care to the first patient. Cefazolin was administered to the second patient for 3 weeks, followed by cephalexin for 1 week. OUTCOMES: The first patient died of a brain hemorrhage and multiple organ failure. The second patient was cured without relapse. LESSONS: Of 18 patients in the previous and current studies with EG caused by S. aureus, 6 (33%) had an underlying hematological malignancy, and 10 (56%) had EG caused by MRSA. While 28% of the patients had positive blood cultures, all tissue cultures were positive. All 3 fatalities had concurrent bacteremia (MRSA caused two). EG caused by MRSA with concurrent bacteremia can be fatal, especially in patients with hematological malignancies. Although S. aureus-associated EG in patients with hematological malignancies is relatively uncommon, tissue cultures with an initial gram stain smear are essential for selecting appropriate empirical antimicrobials, including the coverage of S. aureus.

Moraxella nonliquefaciens-Associated Ecthyma Gangrenosum in a Pediatric Patient With Cancer.

ABSTRACT: In this brief report, we describe a 16-year-old patient with pre-B-cell acute lymphoblastic leukemia on chemotherapy who presented to the emergency department with a fever and "bruise-like" area on his left forearm. Empiric antibiotic therapy was initiated, and initial tissue biopsy demonstrated findings consistent with ecthyma gangrenosum. On day 4 of admission, initial blood cultures grew Moraxella nonliquefaciens, and targeted antibiotic therapy was initiated and continued for a total of 21 days. The patient was discharged after 6 days of in-patient therapy and made a full recovery. M. nonliquefaciens has been reported to be associated with multiple types of infection, but no cases of M. nonliquefaciens-associated ecthyma gangrenosum were identified in the literature review for this report. Given this unique case and the empiric risks and broad differential associated with cutaneous manifestations in immunocompromised patients, obtaining a skin biopsy for histological examination is imperative for diagnostic workup.

Ecthyma Gangrenosum in Children With Cancer: Diagnosis at a Glance: A Retrospective Study From the Infection Working Group of Italian Pediatric Hematology Oncology Association.

BACKGROUND: To depict ecthyma gangrenosum (EG) clinical presentation and evolution in a large multicenter pediatric retrospective collection of children with malignancies or bone marrow failure syndromes, to facilitate early diagnosis. METHODS: EG episodes diagnosed in the period 2009-2019 were identified by a retrospective review of clinical charts at centers belonging to the Italian Pediatric Hematology Oncology Association. RESULTS: Thirty-eight cases of EG occurring in children (male/female 16/22; median age 5.2 years) with hematologic malignancy (29), allogeneic stem cell transplantation (2) or relapsed/refractory solid tumor (3) were collected. The involved sites were: perineal region (19), limbs (10), trunk (6), head and the iliac crest (3). Bacteremia was present in 22 patients. Overall, the germs isolated were Pseudomonas aeruginosa (30), Stenotrophomonas maltophilia (3) and Escherichia coli (1); 31% of them were multidrug-resistant. All patients received antibacterial treatment, while surgery was performed in 24 patients (63.1%). Predisposing underlying conditions for EG were severe neutropenia (97.3%), corticosteroid treatment (71%) and iatrogenic diabetes (23.7%). All patients recovered, but EG recurred in 5 patients. Nine patients (24%) showed sequelae (deep scars, with muscle atrophy in 2). Four patients (10.5%) died, 1 due to relapse of EG with Carbapenem-resistant Enterobacteriaceae co-infection and 3 due to the progression of the underlying disease. CONCLUSIONS: EG requires early recognition and a proper and timely treatment to obtain the recovery and to avoid larger necrotic lesions, eventually evolving in scarring sequelae.

An Eschar-like souvenir from a journey to Colombia: Ecthyma gangrenosum as a differential diagnosis of tropical diseases in immunocompromised patients - a case report.

BACKGROUND: Ecthyma gangrenosum (EG) is a cutaneous infectious disease characterized by eschar-like skin ulcers typically caused by Pseudomonas aeruginosa. Here, we report a case of relapsing EG in a patient who had returned from a trip to Colombia, thus establishing EG as an important differential diagnosis of tropical diseases, and demonstrating that even long-term antibiotic treatment can result in only partial remission of EG. CASE PRESENTATION: A 77-year-old man with underlying chronic lymphocytic leukemia (CLL) on ibrutinib treatment was admitted because of a superinfected mosquito bite on the left ear and multiple partially necrotic skin lesions disseminated all over the entire body five days after returning from a trip to Colombia. The initial clinical suspicion of a tropical disease (leishmaniosis, systemic mycosis, or others) could not be confirmed. During the diagnostic workup, microbiological cultures of the skin biopsies and bronchoalveolar lavage revealed Pseudomonas aeruginosa, leading to a diagnosis of EG. Initial antibiotic treatment resulted in partial remission. However, the patient had to be re-admitted due to a relapse 3-4 weeks after the first episode. Finally, the patient was successfully treated with a combined approach consisting of antibiotics, recurrent surgical incisions, and administration of immunoglobulins. CONCLUSIONS: In conclusion, EG should be considered as a differential diagnosis in immunosuppressed patients presenting with eschar-like skin ulcers. A combined treatment approach seems to be the best choice to achieve clinical cure and avoid relapse.

Ectima gangrenoso, manifestación cutánea infrecuente de una infección por diversos agentes / Ecthyma gangrenosum, a rare cutaneous manifestation of infection by various agents

El ectima gangrenoso es un trastorno infeccioso infrecuente clásicamente relacionado a bacteriemia, descrito principalmente en poblaciones inmunodeprimidas. El agente más comúnmente relacionado es Pseudomonas aeruginosa, sin embargo, se han descrito otras etiologías bacterianas, hongos filamentosos y levaduras. Su patogénesis está dada por la invasión de la pared de los vasos sanguíneos, causando trombosis arterial y venosa, desencadenando necrosis de epidermis y tejidos subdérmicos. Clínicamente, se manifiesta como máculas, vesículas o pústulas hemorrágicas que evolucionan a úlceras de superficie necrótica rodeadas por un halo eritematoso característico, habitualmente en un contexto clínico de sepsis. El diagnóstico de este cuadro es clínico, sin embargo, el estudio microbiológico es clave en la identificación del agente etiológico y un posterior tratamiento dirigido. En su manejo es esencial una alta sospecha clínica y el inicio de tratamiento antibiótico en forma precoz. La duración del tratamiento es variable y en algunos casos debe asociarse a debridación quirúrgica. El pronóstico es variable dependiendo de múltiples factores: estado inmunológico, agente etiológico, presencia de sepsis y el tiempo de inicio de tratamiento.(AU)

Ecthyma gangrenosum is an infrequent infectious disorder classically related to bacteremia, described mainly in immunosuppressed populations. The most related agent is Pseudomonas aeruginosa, however other bacterial etiologies, filamentous fungi and yeasts have been described. Its pathogenesis is given by the invasion of the blood vessel wall, causing arterial and venous thrombosis, triggering necrosis of epidermis and subdermal tissues. Clinically, it manifests as hemorrhagic macules, vesicles or pustules that evolve into ulcers with a necrotic surface surrounded by a characteristic erythematous halo, usually in a clinical setting of sepsis. The diagnosis of this condition is clinical, however the microbiological study is key in the identification of the etiological agent and a subsequent directed treatment. In its management, a high clinical suspicion and an early start of antibiotic treatment are essential. The duration of treatment is variable and in some cases it must be associated with surgical debridement. The prognosis is variable depending on multiple factors: immunological status, etiological agent, presence of sepsis and time of initiation of treatment.(AU)

Ecthyma Gangrenosum: Vulvar Ulcers, Pseudomonas, and Pancytopenia: A Case Report of an 18-Month-Old Female Toddler.

BACKGROUND: An 18-month-old female toddler presented with severe vulvar ulcers and pancytopenia with investigations revealing Pseudomonas aeruginosa bacteremia. CASE: A previously healthy 18-month-old female toddler presented with 6 days of fevers, vulvar rash, and ulcers. Vulvar cultures showed Staphylococcus aureus and P aeruginosa. Bloodwork showed pancytopenia and P aeruginosa bacteremia. She started receiving broad-spectrum antibiotics. Bone marrow aspirate revealed a hypocellular marrow with erythroid dysplasia. Vulvar ulcers progressed rapidly, therefore magnetic resonance imaging was performed to rule out necrotizing fasciitis. She was diagnosed with ecthyma gangrenosum (EG). Three months after initial presentation, she was diagnosed with precursor B-cell acute lymphoblastic leukemia. SUMMARY AND CONCLUSION: This case highlights that health care providers should suspect EG when severe vulvar ulcers are present with P aeruginosa infection and neutropenia. Because EG poses significant morbidity and mortality, its presence should prompt aggressive antimicrobial therapy and mobilization of a multidisciplinary team to initiate workup for an underlying immunodeficiency syndrome or malignancy. This case also illustrates that surgical debridement might be avoided in certain patients with EG as long as meticulous wound care and close monitoring with a multidisciplinary team are in place.

Ecthyma gangrenosum: a report of eight cases

Abstract: Ecthyma gangrenosum is a rare skin infection classically associated with Pseudomonas aeruginosa. We performed a retrospective study of all cases diagnosed with ecthyma gangrenosum from 2004-2010 in a university hospital in Mexico (8 cases, 5 female patients and 3 male patients, ages between 4 months and 2 years). The most common risk factor for ecthyma gangrenosum is neutropenia in immunocompromised patients. In previously healthy patients, immunological evaluation is important to rule out underlying immunodeficiency. Ecthyma gangrenosum in healthy patients has a high mortality rate and early diagnosis and aggressive antibiotic treatment is imperative as it can improve patients' prognosis.

Ecthyma gangrenosum caused by Citrobacter freundii.

A 55-year-old man undergoing chemotherapy for recurrent multiple myeloma presented with a 2-day history of bilateral lower leg rash with pain and oedema. On examination, there were numerous non-palpable retiform pruritic patches over both lower legs. Skin pnch biopsy demonstrated a diffuse interstitial neutrophilic infiltrate with necrosis. Peripheral blood and skin tissue cultures both isolated Citrobacterfreundii, consistent with a rare form of ecthyma gangrenosum. The patient responded with appropriate antibiotic therapy and removal of medical port. He made a full recovery from this infectious complication of his underlying immunosuppression.

[Ecthyma gangrenosum caused by Staphylococcus aureus]. / Ectima gangrenoso secundario a Staphylococcus aureus.

Ecthyma gangrenosum is an uncommon necrotizing vasculitis, in most cases secondary to sepsis by Pseudo-mona aeruginosa in immunocompromised patients. However, there have been several reports of ecthyma gangre-nosum caused by other infectious etiologies. We report an unusual case of ecthyma gangrenosum associated with methicillin-resistant Staphylococcus aureus infection in a patient without the classic immunological risk factors described in the literature.

Ectima gangrenoso secundario a Staphylococcus aureus / Ecthyma gangrenosum caused by Staphylococcus aureus

El ectima gangrenoso es una vasculitis necrosante poco frecuente, en la mayoría de los casos secundaria a sepsis por Pseudomonas aeruginosa en pacientes inmunocomprometidos. Sin embargo, existen reportes de ectima gangrenoso secundarios a otras etiologías infecciosas. Presentamos un caso de ectima gangrenoso asociado a una infección por Staphylococcus aureus resistente a meticilina en una paciente sin los factores de riesgo clásicos de inmunosupresión que se describen en la literatura médica.

Ecthyma gangrenosum is an uncommon necrotizing vasculitis, in most cases secondary to sepsis by Pseudo-mona aeruginosa in immunocompromised patients. However, there have been several reports of ecthyma gangre-nosum caused by other infectious etiologies. We report an unusual case of ecthyma gangrenosum associated with methicillin-resistant Staphylococcus aureus infection in a patient without the classic immunological risk factors described in the literature.

Atypical case of ecthyma gangrenosum mimicking a breast cancer recurrence.

A 68-year-old woman with a history of breast cancer presented with skin lesions and sternal pain. Clinical examination revealed ulcerative lesions on mastectomy scar and CT scan showed contiguous sternal osteolysis. The main hypothesis was a breast cancer recurrence; however, cutaneous and bone biopsies did not reveal any cancer cells. Skin tissue culture detected Pseudomonas aeruginosa, suggesting ecthyma gangrenosum despite the absence of bacteraemia in our patient and despite her immunocompetence. Surgical treatment was performed, followed by a long course of antibiotherapy.

[Ecthyma gangrenosum associated with infection involving a methicillin-sensitive, Panton-Valentine-negative strain of Staphylococcus aureus]. / Ecthyma gangréneux dû à une souche de Staphylococcus aureus sensible à la méthicilline et non sécrétrice de toxine de Panton-Valentine.

BACKGROUND: Ecthyma gangrenosum (EG) is an anatomoclinical syndrome commonly associated with Pseudomonas aeruginosa cutaneous infection. Other microorganisms have also been incriminated on occasion, with other viral, fungal and bacterial agents potentially causing EG. In this report, we present an extremely rare case of an EG caused by methicillin-sensitive Staphylococcus aureus (MSSA) infection. This case, highly characteristic of EG both clinically and histologically, calls into question the physiopathological mechanisms of the disease and provides a reminder that it may be caused by a variety of organisms. PATIENTS AND METHODS: A 62-year-old woman, followed for HIV seropositivity at the AIDS stage, developed a painful purpuric skin rash evolving towards necrotic nodules characteristic of ecthyma gangrenosum. Skin biopsy confirmed the diagnosis of EG due to methicillin-sensitive S. aureus (MSSA) infection without toxins or bacteraemia. DISCUSSION: To the best of our knowledge, this is the first case in the literature in which MSSA is reported as the underlying cause of such lesions.