Extensive perineal ecthyma gangrenosum in leukocyte adhesion deficiency type 1 associated with Staphylococcus hominis bacteremia.

Leukocyte adhesion deficiency (LAD), a disorder of neutrophil function, is characterized by a defect in leukocyte adhesion to the endothelium. Recurrent infections in the skin, soft tissue, gingiva, and lungs due to Staphylococcus aureus, Pseudomonas aeruginosa, and Klebsiella sp. are common in these patients. Ecthyma gangrenosum (EG) is an ulcer of skin and subcutaneous tissue with a black eschar and surrounding erythematous halo secondary to a bacterial infection. Here, we report an unusual presentation of LAD type-1 with extensive EG of perineum secondary to Staphylococcus hominis bacteremia treated successfully with combination of granulocyte transfusion and diversion colostomy.

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.

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.

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.

Ecthyma gangrenosum: The critical role of biofilms and other mechanisms of antibiotic resistance and implications for management.

Ecthyma gangrenosum is a rare cutaneous infection that occurs classically in immunocompromised patients with Pseudomonas aeruginosa bacteremia and is associated with a high mortality rate. Causative pathogens may exhibit various antibiotic evasion mechanisms, and thus, treatment may be challenging. We present a case of ecthyma gangrenosum in association with an implantable port in which cultures confirmed ten unique strains of Pseudomonas aeruginosa, highlighting the ability of this pathogen to form biofilms, rapidly mutate and ultimately evade antibiotic therapy. Dermatologists play a key role in the prompt diagnosis of this life-threatening condition, and a thorough understanding of pathogenic mechanisms is critical in selecting an efficacious treatment regimen.

Ecthyma gangrenosum without bacteremia in a 54-year-old woman with heart transplant.

Ecthyma gangrenosum is a cutaneous manifestation of severe systemic pseudomonas infection and is commonly associated with bacteremia. It is common in immunocompromised patients with underlying neutropenia or leukopenia. We report a patient with heart transplant who developed a single lesion of ecthyma gangrenosum in the absence of bacteremia with a review of reported cases in solid organ transplant patients.

Ecthyma gangrenosum due to Pseudomonas aeruginosa sepsis as initial manifestation of X-linked agammaglobulinemia: a case report.

BACKGROUND: X-linked agammaglobulinemia (XLA, OMIM#300,300), caused by mutations in the Bruton tyrosine kinase (BTK) gene, is a rare monogenic inheritable immunodeficiency disorder. Ecthyma gangrenosum is a cutaneous lesion caused by Pseudomonas aeruginosa that typically occurs in patients with XLA and other immunodeficiencies. CASE PRESENTATION: We report the case of a 20-month-old boy who presented with fever, vomiting, diarrhea, and ecthyma gangrenosum. Blood, stool, and skin lesion culture samples were positive for P. aeruginosa. A diagnosis of XLA was established, and the c.262G > T mutation in exon 4 of BTK was identified with Sanger sequencing. Symptoms improved following treatment with antibiotics and immunoglobulin infusion. CONCLUSIONS: Primary immunodeficiency (i.e., XLA) should be suspected in male infants with P. aeruginosa sepsis, highlighting the importance of genetic and immune testing in these patients.

Ibrutinib-associated sever skin toxicity: A case of multiple inflamed skin lesions and cellulitis in a 68-year-old male patient with relapsed chronic lymphocytic leukemia - Case report and literature review.

INTRODUCTION: Ibrutinib is an oral inhibitor of Bruton's tyrosine kinase that is used for a variety of B cell hematological malignancies. Skin and subcutaneous tissue manifestations have been reported and were witnessed in up to 32% of the patients on ibrutinib. The mechanism in which ibrutinib can cause skin toxicities has been thought due to the inhibition of epidermal growth factor; c-Kit and platelet-derived growth factor receptor). Here, we report a case of an elderly chronic lymphocytic leukemia patient who developed multiple inflamed lesions and lower limb cellulitis in 100 days after initiating ibrutinib therapy. CASE REPORT: A 68-year-old male patient with relapsed chronic lymphocytic leukemia was started on ibrutinib 420 mg orally daily following multiple lines of therapy. Three months following ibrutinib, the patient developed multiple hyper pigmented lesions over both forearms then over both thighs; buttocks and lower limbs. The lesions were labeled as ecthyma and cellulitis that started as papules, which progressed to pustules. MANAGEMENT AND OUTCOMES: The patient required admission in which he received prolonged course of antibiotics. Biopsy from the wound showed soft tissue fragment infiltrated by acute and chronic inflammatory cells with necrosis; rare foreign body giant cells and granulation tissue formation; suggestive of abscess. Subsequently, ibrutinib was stopped permanently. DISCUSSION: This is the first case description of an ibrutinib-associated sever skin toxicity in Qatar. The provided information regarding the clinical descriptions of toxicity profiles in general and skin-based in particular is valuable information for daily clinical practice, especially when selecting the optimum first-line treatment for the patient.

Hairy cell leukemia presenting with Ecthyma Gangrenosum- a case report.

BACKGROUND: Ecthyma gangrenosum is a cutaneous infectious usually associated with P. aeruginosa. It usually develops In patients with an underlying immunodeficiency. CASE PRESENTATION: A 50-year old mentally disabled white male with a history of epilepsy presented with fever and a painless red macule on his right arm which rapidly progressed to a painful ulcer. Blood and lesion cultures revealed P.aeruginosa, confirming our clinical diagnosis of ecthyma gangrenosum. Subsequently an underlying immune deficit was found, namely patient was diagnosed with hairy-cell leukemia. Despite adequate antibiotics no infection control could be achieved. After treating the underlying immune deficit as well, the infection and hairy-cell leukemia resolved completely. CONCLUSION: Ecthyma gangrenosum is an important cutaneous infection to recognize, because it is it is typically associated with P.aeruginosa bacteremia. Recognizing this skin leasion should prompt empiric antimicrobial therapy including an agent with antipseudomonal activity. Furthermore, just like in our case, the presence of ecthyma gangrenosum can signal the presence of an occult immune deficit, warranting further investigation.

Ecthyma gangrenosum in a 3-year-old boy post-heart transplantation.

Ecthyma gangrenosum (EG) is a serious bacterial infection in immunocompromised patients. EG in transplant recipients is rarely reported and may go unrecognized, which may delay initiation of appropriate treatment. We report a case of EG in a pediatric heart transplant recipient who was treated successfully with antibiotics and surgical debridement.

Utility of platelet rich fibrin gel therapy in nonhealing ulcer secondary to ecthyma gangrenosum.

Platelet rich fibrin (PRF) gel is a new second generation platelet concentrate, which has been widely used in various dermatological conditions such as nonhealing diabetic foot ulcers, venous ulcers, and trophic ulcers in leprosy. In this case report, we present a patient with nonhealing ulcer secondary to ecthyma gangrenosum, who showed dramatic response with PRF gel. This case suggests a role of PRF gel in nonhealing ulcers of infectious etiology which has not been described in English language literature. Considering previous literature and our case, it may be concluded that PRF gel may be utilized as an effective alternative for nonhealing ulcers of nonneoplastic etiology.

Juvenile ecthyma gangrenosum caused by Pseudomonas aeruginosa revealing an underlying neutropenia: case report and review of the literature.

BACKGROUND: Ecthyma gangrenosum (EG) is characterized by the occurrence of erythematous, violaceous or haemorrhagic macules and/or vesicles, often evolving into necrotic ulcers, with a central grey-black eschar. It is a rare skin condition, usually occurring in immunocompromised patients suffering from bacterial sepsis caused by Pseudomonas aeruginosa. However, seemingly healthy children have been diagnosed with this skin disease as well. 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. Moreover, we provide a brief literature review on those cases of EG where an underlying primary immunodeficiency, neutropenia in particular, was eventually diagnosed. 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. Workup consisted of microbiological and haematological investigations, including repeated blood analyses. RESULTS: Bacterial swabs from the vulvar ulcers showed the growth of P. aeruginosa. No concomitant sepsis was present, but laboratory investigations pointed towards a cyclic neutropenia, coinciding with the occurrence of the EG lesions. Topical gentamicin ointment allowed the skin lesions to heal faster. Following the administration of granulocyte colony-stimulating factor (G-CSF), the girl experienced less infections in general and had no recurrence of EG lesions in particular. Treatment with G-CSF could eventually be stopped, and the neutropenia, ultimately transient in nature, completely resolved. CONCLUSION: Children presenting with (anogenital) EG should always alert a physician to consider a potentially underlying immunodeficiency, neutropenia in particular.

[Multiple well demarcated skin erosions and ulcers following exanthematous drug eruption after sultamicillin therapy]. / Scharfrandige Erosionen und Ulzerationen im Anschluss an ein Arzneimittelexanthem nach Sultamicillin-Gabe.

This article presents the case of a patient with newly developed skin erosions and ulcerations following an exanthematous drug eruption due to sultamicillin therapy. The skin lesions were treated topically with clobetasol and prednicarbate and orally with methylprednisolone. A skin smear revealed massive growth of Escherichia coli bacteria. Blood cultures were negative. The cause for developing ecthyma gangrenosum in our patient were iatrogenic immunosuppression and transient bacteremia.

Ecthyma gangrenosum without bacteraemia: evidence in favour of a broader definition.

Ecthyma gangrenosum (EG) is often defined as a cutaneous manifestation of Pseudomonas aeruginosa septicaemia, typically secondary to neutropenia. There is increasing recognition that a broader definition is warranted, as numerous causative organisms and predisposing conditions have been reported. We describe two cases of EG that occurred without bacteraemia. In this atypical subset of cases, the skin is thought to represent the primary inoculation site from which haematogenous spread can occur. The first case occurred in the context of human immunodeficiency virus (HIV) infection, a rarely reported association. The evidence base guiding clinicians on management of EG is very limited, particularly in relation to patients with HIV, in whom recurrence risk may be higher than normal. Recurrent EG is described in our second case, in an individual with Good syndrome, a rare association of thymoma and immunodeficiency.

Pasteurella multocida ecthyma complicated by necrotizing fasciitis.

Necrotizing fasciitis is a serious infection of the skin and soft tissues. Pasteurella multocida is rarely reported to cause necrotizing fasciitis and is associated with high mortality. We describe a female patient with a past medical history of diabetes mellitus and myeloproliferative disorder presenting with bullae and erythema of the right forearm secondary to P. multocida infection after possible cat bite. Despite adequate antibiotic coverage she developed necrotizing fasciitis diagnosed clinically and on diagnostic imaging. Patient was taken to the operating room emergently and underwent irrigation and debridement with subsequent split-skin graft. She recovered well after the surgeries and was discharge on intravenous antibiotics. At clinic follow-up, her wounds were healing well without any significant new symptoms.