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

Huang, Hai-Xia; Bai, Ke; Fu, Yan; Jin, Yan; Li, Jing

doi:10.1186/s12887-020-02436-8

Cited by 3 publications

(1 citation statement)

References 14 publications

(27 reference statements)

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“…Similar to our earlier observations and those reported from elsewhere ( 4 – 9 , 12 ), Staphylococcus aureus abscess formation was a consistent feature in HIGE syndrome and DOCK8 deficiency patients. Besides the common staphylococcal skin infections, patients are also prone to get rare infections due to Gram-negative bacteria that may result in EG, which has been reported earlier in patients with X-linked agammaglobulinemia, chronic granulomatous disease, and hemophagocytic lymphohistiocytosis ( 13 – 15 ).…”

Section: Discussionmentioning

confidence: 90%

A Prospective Survey of Skin Manifestations in Children With Inborn Errors of Immunity From a National Registry Over 17 Years

Al‐Herz

Zainal

Nanda³

2021

Front. Immunol.

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Background and ObjectivesReports on skin manifestations in inborn errors of immunity (IEI) are based on retrospective analysis, small series, or isolated case reports. The present prospective study aimed to determine the spectrum of skin manifestations in children with IEI and their relevance to specific molecular defects.Materials and MethodsThe data were obtained from the Kuwait National Primary Immunodeficiency Disorders Registry during the period of 2004–2020.ResultsA total of 313 pediatric cases of IEI, 71% diagnosed at molecular level, were registered with a cumulative follow-up period of 29,734 months. Skin manifestations were seen in 40.3% of the patients, and they were among the presenting manifestations in 33%. Patients with skin manifestations were older at both onset and diagnosis ages of IEI symptoms, but this was statistically significant for the latter only. The diagnosis delay was significantly longer in patients with skin manifestations. There was a statistically significant association between having skin manifestations and IEI category, being more common in patients with complement deficiencies, combined immunodeficiencies, and diseases of immune dysregulation. There was no statistically significant association between having skin manifestations and both gender and survival. Skin infections were the most frequent manifestations followed by eczema and autoimmune associations. Among IEI with more than 10 cases, skin lesions were a consistent finding in dedicator of cytokinesis 8 (DOCK8) deficiency, hyper IgE syndrome, ataxia-telangiectasia, and recombination activation gene (RAG)1 deficiency.ConclusionsSkin manifestations are common in IEI patients, and they had significant diagnosis delay and referral to specialists. Improvement of awareness about IEI is needed among pediatricians and dermatologists.

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Section: Discussionmentioning

confidence: 90%

A Prospective Survey of Skin Manifestations in Children With Inborn Errors of Immunity From a National Registry Over 17 Years

Al‐Herz

Zainal

Nanda³

2021

Front. Immunol.

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Skin manifestations of Pseudomonas aeruginosa infections

Spernovasilis

Psichogiou

Poulakou

2021

Current Opinion in Infectious Diseases

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Purpose of review Pseudomonas aeruginosa is an opportunistic pathogen with considerable morbidity and mortality, particularly in vulnerable hosts. Skin manifestations are common, either representing local inoculation or secondary skin seeding following bloodstream infections. As patients with various predisposing conditions are expanding, we sought to review the most recent published evidence regarding epidemiology, risk factors and diagnosis of skin manifestations of P. aeruginosa. Recent findings New data exist on epidemiology and diagnosis of skin infections; systemic infections are impacted by multidrug-resistance issues and host immune status. Summary Green nail syndrome, toe web infection, hot tub folliculitis, hot hand-foot infection and external otitis are the most common infections originating from the skin per se. Local treatments are the cornerstone and prognosis is favorable in immunocompetent hosts. Ecthyma gangrenosum and P. aeruginosa subcutaneous nodules are usually associated with bloodstream infections and occur primarily in immunocompromised hosts. Necrotizing skin and soft tissue infections occur in diabetic, alcoholic and immunocompromised patients; management requires a multidisciplinary team with surgical approach. Burn wound infections may also be challenging, requiring a specialized team. In all the four latter types of P. aeruginosa skin infections portending significant morbidity and mortality, systemic antibiotics are an integral part of the treatment.

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Novel evidence on sepsis-inducing pathogens: from laboratory to bedside

Gatica,

Fuentes,

Rivera-Asín

et al. 2023

Front. Microbiol.

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Sepsis is a life-threatening condition and a significant cause of preventable morbidity and mortality globally. Among the leading causative agents of sepsis are bacterial pathogens Escherichia coli, Klebsiella pneumoniae, Staphylococcus aureus, Pseudomonas aeruginosa, and Streptococcus pyogenes, along with fungal pathogens of the Candida species. Here, we focus on evidence from human studies but also include in vitro and in vivo cellular and molecular evidence, exploring how bacterial and fungal pathogens are associated with bloodstream infection and sepsis. This review presents a narrative update on pathogen epidemiology, virulence factors, host factors of susceptibility, mechanisms of immunomodulation, current therapies, antibiotic resistance, and opportunities for diagnosis, prognosis, and therapeutics, through the perspective of bloodstream infection and sepsis. A list of curated novel host and pathogen factors, diagnostic and prognostic markers, and potential therapeutical targets to tackle sepsis from the research laboratory is presented. Further, we discuss the complex nature of sepsis depending on the sepsis-inducing pathogen and host susceptibility, the more common strains associated with severe pathology and how these aspects may impact in the management of the clinical presentation of sepsis.

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

Cited by 3 publications

References 14 publications

A Prospective Survey of Skin Manifestations in Children With Inborn Errors of Immunity From a National Registry Over 17 Years

A Prospective Survey of Skin Manifestations in Children With Inborn Errors of Immunity From a National Registry Over 17 Years

Skin manifestations of Pseudomonas aeruginosa infections

Novel evidence on sepsis-inducing pathogens: from laboratory to bedside

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