Neutrophilic dermatosis and hidradenitis suppurativa in patients with Behçet's disease: A neutrophilic disease in the spectrum of autoinflammatory syndromes.
• Pyoderma gangrenosum seems overrepresented in patients with Behçet's disease. • The clinical course of neutrophilic dermatosis may not correlate with Behçet's disease activity and treatment may be challenging. • Biotherapies such as anti-TNFα, ustekinumab and tocilizumab appear to be promising to...
| Publicado en: | Seminars in Arthritis & Rheumatism Vol. 61 |
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| Autores principales: | , , , , , , , , , , |
| Formato: | research Journal Article |
| Publicado: |
W B Saunders
Aug2023
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=164863298&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 164863298 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 00490172 4GQ jtl: Seminars in Arthritis & Rheumatism issn: 00490172 maglogo: N pubinfo: dt: Aug2023 vid: 61 pid: 1351 pub: W B Saunders place: Philadelphia, Pennsylvania artinfo: ui: 164863298 164863298 164863298 10.1016/j.semarthrit.2023.152224 164863298 ppct: 1 formats: tig: atl: Neutrophilic dermatosis and hidradenitis suppurativa in patients with Behçet's disease: A neutrophilic disease in the spectrum of autoinflammatory syndromes. aug: au: Bugaut, Hélène Barete, Stéphane Bagot, Martine Bouaziz, Jean-David Le Pelletier de Glatigny, François Gallien, Yves Biard, Lucie Domont, Fanny Cacoub, Patrice Saadoun, David Comarmond, Cloé affil: Department of Internal Medicine and Clinical Immunology, Sorbonne University, Pitié-Salpêtrière Hospital, APHP, 75013 Paris France, Centre de référence Maladies Autoimmunes systémiques rares, Centre de référence Maladies Autoinflammatoires et amylose, 83 boulevard de l'hôpital, Paris 75013, France sug: subj: Sweet's Syndrome Complications Hidradenitis Suppurativa Complications Behcet's Syndrome Risk Factors Hereditary Autoinflammatory Diseases Human Pyoderma Gangrenosum Biological Therapy Colchicine Glucocorticoids ab: • Pyoderma gangrenosum seems overrepresented in patients with Behçet's disease. • The clinical course of neutrophilic dermatosis may not correlate with Behçet's disease activity and treatment may be challenging. • Biotherapies such as anti-TNFα, ustekinumab and tocilizumab appear to be promising to treat refractory neutrophilic dermatosis and hidradenitis suppurativa associated with Behçet's disease. Association of neutrophilic dermatosis (ND), hidradenitis suppurativa (HS) and Behçet's disease (BD) and shared efficacy of TNFα axis blockade suggests common physiopathology. To investigate the clinical features and therapeutic response of ND and HS associated with BD. We identified 20 patients with ND or HS associated with BD among 1462 patients with BD. 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. 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. [Display omitted] pubtype: Academic Journal doctype: research Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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