Autoinflammatorische Dermatosen

Ehrchen, Jan; Drerup, Christian

doi:10.1007/s00105-020-04579-2

Cited by 4 publications

(2 citation statements)

References 24 publications

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“…Therefore, it has also been confirmed that such diseases are a result of innate immunity dysfunction through most of their responsiveness to IL‐1β blockade, which indicates the key pro‐inflammatory cytokine of innate immune reactions 10 . In contrast to autoimmune diseases, which are activated by adaptive immune cells, no specific autoantibodies or autoreactive immune cells are located in autoinflammatory diseases 11 …”

Section: Skin Autoinflammatory Diseasesmentioning

confidence: 98%

“…10 In contrast to autoimmune diseases, which are activated by adaptive immune cells, no specific autoantibodies or autoreactive immune cells are located in autoinflammatory diseases. 11 The pathogenesis of autoinflammatory diseases largely involves the dysregulation of inflammasomes that leads to excessive production of interleukin 1β. The diagnosis of such diseases requires recognition of the trends in presentation and differential diagnosis.…”

Section: S K In Autoinfl Ammatory D Is E a S E Smentioning

confidence: 99%

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Molecular analysis of inflammatory diseases

Almughrbi

Crovella

2022

Experimental Dermatology

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If we try to describe the search for molecular actors involved in inflammatory diseases, the picture best representing this task is a mission to unexplored worlds. However, researchers nowadays have powerful tools to support this journey to the complexity of the unknown: Next generation Sequencing technologies have provided a plethora of data describing the different OMICs possibly involved in the different inflammatory diseases. Here, we focused on autoinflammatory skin diseases showing the progress of OMICs‐related findings in the understanding of Syndromic HS pathogenesis. We described the studies reporting possible genotype/phenotype correlation in PASH and PAPASH patients (both unrelated or familial cases), highlighting those just genetic variations associated with the diseases have been observed, but the information on common pathways shared by PASH and PAPASH patients were lacking, thus rendering difficult to decipher the common molecular basis of these autoinflammatory conditions. Aimed at filling this gap of knowledge, we proposed an integrated OMICs approach able to identify common pathways shared by subjects suffering from PASH and PAPASH: pathway‐based whole sequencing analysis allowed the identification of 4 pathways, keratinization, formation of the cornified envelope steroid metabolism and Vitamin D metabolism, disrupted in PASH and PAPASH patients. Finally, we mentioned the novel bioinformatic platform, named PlatOMICs, capable of integrating OMICs experimental findings also with the ones already reported in public repositories supporting the efforts of the researchers and clinicians to discover molecular pathways shared by individuals suffering of a disease, confronting and integrating the bench findings with the in‐silico ones.

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Section: Skin Autoinflammatory Diseasesmentioning

confidence: 98%

Section: S K In Autoinfl Ammatory D Is E a S E Smentioning

confidence: 99%

Molecular analysis of inflammatory diseases

Almughrbi

Crovella

2022

Experimental Dermatology

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Uso de la lipotransferencia en el tratamiento de las alteraciones cutáneas por alogenosis iatrogénica

Carbón

Trujillo

Figueroa

et al. 2022

Piel

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Pyoderma Gangrenosum after Cardiac Surgery

Petrov¹,

Kappert²,

Schmidt

et al. 2021

Thorac Cardiovasc Surg

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Background Pyoderma gangrenosum after cardiac surgery is a rare, noninfectious ulcerating skin disease mimicking sternal wound infection. Methods A systematic search of literature for pyoderma gangrenosum complicating cases of cardiac surgery was conducted between September 1985 and September 2020 on PubMed and Cochrane databases. A systematic review and detailed overview of clinical presentation, diagnostic, treatment, and outcome is provided. Results A total of 15 studies enclosing 15 patients suffering from pyoderma gangrenosum following cardiac surgery were identified. Onset of symptoms was observed after a median of 5 days. Patients were predominantly male (81.3%) with a median age of 64 years. Typical clinical presentation mimicked sternal site infection, mainly by means of mediastinitis. Specific signs were rapid progression, erythematous to violaceous color of the wound border, accompanied by unspecific symptoms including fever, malaise, and severe pain. Additionally, pathergy (development of ulcers at the sites of minor cutaneous trauma) was reported frequently. Biopsy is mandatory with a cutaneous neutrophilic inflammation confirming the diagnosis. Initial treatment mostly (75.0% of reported cases) was misled, addressing suspicion of surgical site infection. After correct diagnosis, the treatment was switched to an immunosuppressive therapy. Full sternal wound closure took between 5 weeks and 5 months. Reported case mortality was 12.5% in actually low-risk surgeries. Conclusion Despite pyoderma gangrenosum has typical signs, it remains an exclusion diagnosis. The treatment is completely opposite to the main differential diagnosis—the typical surgical site infection. Knowledge about diagnosis and treatment is essential in the context of avoiding fatal mistreatment.

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Autoinflammatorische Dermatosen

Cited by 4 publications

References 24 publications

Molecular analysis of inflammatory diseases

Molecular analysis of inflammatory diseases

Uso de la lipotransferencia en el tratamiento de las alteraciones cutáneas por alogenosis iatrogénica

Pyoderma Gangrenosum after Cardiac Surgery

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