Phenotypes and Pathophysiology of Syndromic Hidradenitis Suppurativa: Different Faces of the Same Disease? A Systematic Review.

Hidradenitis Suppurativa / diagnosis Humans
Acne inversa Arthritis Autoinflammatory disorder Hidradenitis suppurativa Inflammatory bowel disease Pustulosis Syndrome Synovitis-acne-pustulosis-hyperostosis-osteitis

Journal

Dermatology (Basel, Switzerland)
ISSN: 1421-9832
Titre abrégé: Dermatology
Pays: Switzerland
ID NLM: 9203244

Informations de publication

Date de publication:
2021
Historique:
received: 23 04 2020
accepted: 27 06 2020
pubmed: 18 9 2020
medline: 27 1 2022
entrez: 17 9 2020
Statut: ppublish

Résumé

There is growing evidence that (certain) hidradenitis suppurativa (HS) comorbidities comprise syndromes including HS as a key cutaneous manifestation. These apparently autoinflammatory syndromes and their diagnostic delay might have detrimental effects on affected patients. A systematic review was performed on the databases MEDLINE, EMBASE, and CENTRAL utilizing a standardized extraction form according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Sixty-four eligible articles on syndromic HS were retrieved. The identified syndromes included already described ones (pyoderma gangrenosum-acne-suppurative hidradenitis, pyogenic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, psoriatic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, pyoderma gangrenosum-acne vulgaris-hidradenitis suppurativa-ankylosing spondylitis, synovitis-acne-pustulosis-hyperostosis-osteitis) and further novel symptom constellations. Cutaneous signs, including HS lesions, usually precede signs from other organs. The cutaneous signs of a considerable proportion of patients appear refractory to conventional treatment, and monotherapy with biologics does not suffice to sustain remission. The results are subsequently discussed with focus on the pathophysiology and treatment of the detected syndromes. The dermatologist's role in the precise diagnosis and early treatment administration of HS is pivotal. The purpose of the treatment should be the effective prevention or delay of the autoinflammatory march and its irreversible consequences.

Sections du résumé

BACKGROUND BACKGROUND
There is growing evidence that (certain) hidradenitis suppurativa (HS) comorbidities comprise syndromes including HS as a key cutaneous manifestation. These apparently autoinflammatory syndromes and their diagnostic delay might have detrimental effects on affected patients.
METHODS METHODS
A systematic review was performed on the databases MEDLINE, EMBASE, and CENTRAL utilizing a standardized extraction form according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
RESULTS RESULTS
Sixty-four eligible articles on syndromic HS were retrieved. The identified syndromes included already described ones (pyoderma gangrenosum-acne-suppurative hidradenitis, pyogenic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, psoriatic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, pyoderma gangrenosum-acne vulgaris-hidradenitis suppurativa-ankylosing spondylitis, synovitis-acne-pustulosis-hyperostosis-osteitis) and further novel symptom constellations. Cutaneous signs, including HS lesions, usually precede signs from other organs. The cutaneous signs of a considerable proportion of patients appear refractory to conventional treatment, and monotherapy with biologics does not suffice to sustain remission.
CONCLUSION CONCLUSIONS
The results are subsequently discussed with focus on the pathophysiology and treatment of the detected syndromes. The dermatologist's role in the precise diagnosis and early treatment administration of HS is pivotal. The purpose of the treatment should be the effective prevention or delay of the autoinflammatory march and its irreversible consequences.

Identifiants

DOI: 10.1159/000509873 PMID: 32942279
pubmed: 32942279
pii: 000509873
doi: 10.1159/000509873
doi:

Types de publication

Journal Article Meta-Analysis Systematic Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

673-697

Informations de copyright

© 2020 S. Karger AG, Basel.

Auteurs

Georgios Nikolakis (G)

Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany, nikolakisgeorgios@gmail.com.
European Hidradenitis Suppurativa Foundation e.V., Dessau, Germany, nikolakisgeorgios@gmail.com.

Katarzyna P Kaleta (KP)

Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany.
Department of Dermatology, Jagiellonian University Medical College, Krakow, Poland.

Aristeidis G Vaiopoulos (AG)

Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany.
European Hidradenitis Suppurativa Foundation e.V., Dessau, Germany.

Katja Wolter (K)

Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany.

Sumer Baroud (S)

Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany.
University of Sharjah, Sharjah, United Arab Emirates.

Anna Wojas-Pelc (A)

Department of Dermatology, Jagiellonian University Medical College, Krakow, Poland.

Christos C Zouboulis (CC)

Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany.
European Hidradenitis Suppurativa Foundation e.V., Dessau, Germany.

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Classifications MeSH

questionsmedicales.fr › Maladies de la peau et du tissu conjonctif › Maladies de la peau › Maladies des glandes sudoripares › Hidrosadénite › Hidrosadénite suppurée › Phenotypes and Pathophysiology of Syndromic...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Phenotypes and Pathophysiology of Syndromic...