Short‐term efficacy of adalimumab in a patient with pyrin‐associated autoinflammation with neutrophilic dermatosis

Gargallo, Vanessa; Menis, Diana; Márquez, Ana María Delgado; Aróstegui, Juan I.; Martin, R. Llamas

doi:10.1111/ddg.13486

Cited by 11 publications

(11 citation statements)

References 8 publications

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“…Due to our case chronology, we suggest that the herpes zoster virus first damaged the folliculosebaceous unit, exposing sebocyte antigens and triggering the autoimmune cascade. SA in our patient could also be considered as neutrophilic dermatosis [12] associated to Crohn's disease; however, the main inflammatory infiltrate in our case was lymphomonocytic.…”

mentioning

confidence: 56%

Sebaceous adenitis in a man with autoimmune background

Herrero‐Moyano

Muñoz‐Aceituno

Fernández‐Figueras

et al. 2020

J Deutsche Derma Gesell

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Sebaceous adenitis (SA) is a rare cutaneous inflammatory disease of unknown origin which was first described by Renfro in 1993 [1]. Diagnosis is based on histopathological features, mainly distended sebaceous gland with sebocyte necrosis, which can be surrounded by a mostly neutrophilic infiltrate. Due to the neutrophilic infiltrate in the first described case, this disease has been also named neutrophilic sebaceous adenitis.A 32-year-old man of Moroccan origin presented with an asymptomatic eruption that started five days before on his face and later progressed to his trunk. He reported mild asthenia and malaise, but no fever, arthralgia or photosensitivity.

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mentioning

confidence: 56%

Sebaceous adenitis in a man with autoimmune background

Herrero‐Moyano

Muñoz‐Aceituno

Fernández‐Figueras

et al. 2020

J Deutsche Derma Gesell

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“…In patients with FMF, the use of canakinumab significantly reduces attack frequency and normalizes inflammatory markers, also in those that are resistant to treatment with NSAIDs and colchicine 248 . However, in patients with MEFV mutations that cause PAAND, case reports have described that treatment with corticosteroids and anakinra is ineffective, while long‐lasting clinical responses were achieved with anti‐TNF drugs (in two cases infliximab for 8 and 10 years, followed up by adalimumab) 213,249 . Supportively, the skin lesions in PAAND more closely resemble those described in the pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome, caused by AD mutations in PSTPIP1 , in which the pyoderma is also successfully treated with anti‐TNF blockade 250 .…”

Section: Host‐directed Treatment Strategies In Immunological Gain‐of‐mentioning

confidence: 99%

Primary immunodeficiencies in cytosolic pattern‐recognition receptor pathways: Toward host‐directed treatment strategies

Made

Hoischen

Netea

et al. 2020

Immunological Reviews

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In the last decade, the paradigm of primary immunodeficiencies (PIDs) as rare recessive familial diseases that lead to broad, severe, and early‐onset immunological defects has shifted toward collectively more common, but sporadic autosomal dominantly inherited isolated defects in the immune response. Patients with PIDs constitute a formidable area of research to study the genetics and the molecular mechanisms of complex immunological pathways. A significant subset of PIDs affect the innate immune response, which is a crucial initial host defense mechanism equipped with pattern‐recognition receptors. These receptors recognize pathogen‐ and damage‐associated molecular patterns in both the extracellular and intracellular space. In this review, we will focus on primary immunodeficiencies caused by genetic defects in cytosolic pattern‐recognition receptor pathways. We discuss these PIDs organized according to their mutational mechanisms and consequences for the innate host response. The advanced understanding of these pathways obtained by the study of PIDs creates the opportunity for the development of new host‐directed treatment strategies.

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“…Die Begründung für die Wirksamkeit eines Anti-TNFα-Wirkstoffs beim refraktären Sweet-Syndrom, insbesondere bei Assoziation mit entzündlichen Darmerkrankungen (IBD), kann durch die positive Wirkung des Medikaments auf die Darmentzündung erklärt werden, selbst im Falle einer klinisch inaktiven IBD. Andererseits kann die Wirksamkeit des Medikaments, die bereits bei anderen neutrophilen Dermatosen [11,12] untersucht wurde, auch auf eine direkte Wirkung der TNFα-Hemmung auf den kutanen Entzündungsweg zurückgeführt warden. So gibt es immer mehr Beweise für die Rolle von TNFα in der Pathophysiologie neutrophiler Dermatosen, einschließlich des Sweet-Syndroms [13].…”

Section: Correspondence Clinical Letterunclassified