Neutrophilic dermatosis and hidradenitis suppurativa in patients with Behçet's disease : A neutrophilic disease in the spectrum of autoinflammatory syndromes
Copyright © 2023 Elsevier Inc. All rights reserved..
BACKGROUND: Association of neutrophilic dermatosis (ND), hidradenitis suppurativa (HS) and Behçet's disease (BD) and shared efficacy of TNFα axis blockade suggests common physiopathology.
OBJECTIVES: To investigate the clinical features and therapeutic response of ND and HS associated with BD.
METHODS: We identified 20 patients with ND or HS associated with BD among 1462 patients with BD.
RESULTS: We analysed 20 (1.4%) patients diagnosed with ND or HS associated with BD: 13 HS, 6 pyoderma gangrenosum (PG), and 1 SAPHO. Our 6 PG cases over 1462 BD patients accounts for 400/100 000 prevalence. Thirteen had bipolar aphthosis, 6 vascular, 5 neurologic, and 4 ocular involvements. All PG occurred on limbs and had typical histology with constant dermal neutrophilic infiltrate. All HS had the classical axillary-mammary phenotype. Sixty-nine percent (69%) of HS were Hurley 1 stage. Treatment consisted mainly in colchicine (n = 20), glucocorticoids (n = 12), and anti-TNFα (n = 9). Interesting results with complete or partial responses were obtained with anti-TNFα (9 cases), ustekinumab (3 cases) and tocilizumab (1 case) to treat refractory ND or HS associated with BD.
CONCLUSION: PG seems overrepresented in patients with BD. Biotherapies such as anti-TNFα, ustekinumab and tocilizumab appear to be promising to treat refractory ND or HS associated with BD.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:61 |
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| Enthalten in: |
Seminars in arthritis and rheumatism - 61(2023) vom: 03. Aug., Seite 152224 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Bugaut, Hélène [VerfasserIn] |
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| Links: |
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| Themen: |
Anti-TNFα |
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| Anmerkungen: |
Date Completed 10.07.2023 Date Revised 02.01.2024 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.semarthrit.2023.152224 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM357099281 |
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| 520 | |a Copyright © 2023 Elsevier Inc. All rights reserved. | ||
| 520 | |a BACKGROUND: Association of neutrophilic dermatosis (ND), hidradenitis suppurativa (HS) and Behçet's disease (BD) and shared efficacy of TNFα axis blockade suggests common physiopathology | ||
| 520 | |a OBJECTIVES: To investigate the clinical features and therapeutic response of ND and HS associated with BD | ||
| 520 | |a METHODS: We identified 20 patients with ND or HS associated with BD among 1462 patients with BD | ||
| 520 | |a RESULTS: We analysed 20 (1.4%) patients diagnosed with ND or HS associated with BD: 13 HS, 6 pyoderma gangrenosum (PG), and 1 SAPHO. Our 6 PG cases over 1462 BD patients accounts for 400/100 000 prevalence. Thirteen had bipolar aphthosis, 6 vascular, 5 neurologic, and 4 ocular involvements. All PG occurred on limbs and had typical histology with constant dermal neutrophilic infiltrate. All HS had the classical axillary-mammary phenotype. Sixty-nine percent (69%) of HS were Hurley 1 stage. Treatment consisted mainly in colchicine (n = 20), glucocorticoids (n = 12), and anti-TNFα (n = 9). Interesting results with complete or partial responses were obtained with anti-TNFα (9 cases), ustekinumab (3 cases) and tocilizumab (1 case) to treat refractory ND or HS associated with BD | ||
| 520 | |a CONCLUSION: PG seems overrepresented in patients with BD. Biotherapies such as anti-TNFα, ustekinumab and tocilizumab appear to be promising to treat refractory ND or HS associated with BD | ||
| 650 | 4 | |a Journal Article | |
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| 700 | 1 | |a Bouaziz, Jean-David |e verfasserin |4 aut | |
| 700 | 1 | |a Le Pelletier de Glatigny, François |e verfasserin |4 aut | |
| 700 | 1 | |a Gallien, Yves |e verfasserin |4 aut | |
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