Evidence map›Paper›PMID 35720358›Full record

ArticleFrontiers in immunology2022

Case Report: Comorbid Hyper-IgD Syndrome and Hidradenitis Suppurativa - A New Syndromic Form of HS? A Report of Two Cases.

Philippe Guillem, Dillon Mintoff, Mariam Kabbani, Elie Cogan, Virginie Vlaeminck-Guillem, Agnes Duquesne, Farida Benhadou

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In one paragraph

Article in Frontiers in immunology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.

0numbers the graph read from it
0cells of the map it votes in
8citing papers in PubMed
2.5field-weighted citation impact, top 11% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

8 citing papers in PubMed, 15 citations in OpenAlex.

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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

7 authors at 6 institutions in 3 countries.

Philippe GuillemDepartment of Surgery, Clinique du Val d'Ouest, Lyon, France.
Dillon MintoffEuropean Hidradenitis Suppurativa Foundation e.V., Dessau, Germany.
Mariam KabbaniDepartment of Dermatology, Hôpital Universitaire Erasme, Université Libre de Bruxelles, Brussels, Belgium.
Elie CoganDepartment of Internal Medicine, Hôpital Universitaire Erasme, Université Libre de Bruxelles, Brussels, Belgium.
Virginie Vlaeminck-GuillemUniversité Claude Bernard Lyon 1, INSERM 1052, CNRS 5286, Centre Léon Bérard, Centre de 9 Recherche en Cancérologie de Lyon, Lyon, France.
Agnes DuquesneDepartment of Paediatric Nephrology, Rheumatology and Dermatology, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Bron, France.
Farida BenhadouResoVerneuil, Paris, France.
Université Libre de Bruxelles · BECentre Hospitalier Interrégional Edith Cavell · BEHospices Civils de Lyon · FRInserm · FRRéseau pour la Transition Énergétique · FRUniversity of Malta · MT

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Hidradenitis Suppurativa (HS) is a chronic suppurative disease of the pilosebaceous unit. The current model of HS pathophysiology describes the condition as the product of hyperkeratinisation and inflammation at the hair follicular unit. Environmental factors (such as smoking and obesity), gender, genetic predisposition, and skin dysbiosis are considered the main pathogenic drivers of the disease. Autoinflammatory syndromes associated with HS are rare but may help to highlight the potential roles of autoinflammation and dysregulated innate immune system in HS. Therefore, it is of major relevance to increase the awareness about these diseases in order to improve the understanding of the disease and to optimize the management of the patients. Herein, we report for the first time, to our knowledge, two clinical cases of Hyper-IgD syndrome-associated HS. Hyper-IgD is an autoinflammatory syndrome caused by a mevalonate kinase deficiency (MKD), a key kinase in the sterol and isoprenoid production pathway. We describe the potentially shared pathophysiological mechanisms underpinning comorbid MKD-HS and propose therapeutic options for the management of these patients.

Indexed as

Hidradenitis SuppurativaMevalonate Kinase DeficiencyComorbidityHumansInflammationSkinSyndromeautoinflammationautoinflammatory keratinization diseasehidradenitis suppurativahyper-IgD syndromemevalonate kinase deficiency

Identifiers

PMID35720358
PMCPMC9204359
OpenAlexW4281782983

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.