Neutrophilic dermatosis and hidradenitis suppurativa in patients with Behçet's disease: A neutrophilic disease in the spectrum of autoinflammatory syndromes

• Published in: Seminars in arthritis and rheumatism Vol. 61; p. 152224
• Main Authors: 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
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-08-2023
• Subjects: Anti-TNFα; Behcet Syndrome - complications; Behcet Syndrome - drug therapy; Behçet's disease; Hidradenitis suppurativa; Hidradenitis Suppurativa - complications; Hidradenitis Suppurativa - drug therapy; Hidradenitis Suppurativa - epidemiology; Humans; Neutrophilic dermatosis; Pyoderma gangrenosum; Pyoderma Gangrenosum - complications; Pyoderma Gangrenosum - drug therapy; Pyoderma Gangrenosum - epidemiology; SAPHO; Tocilizumab; Ustekinumab; Ustekinumab - therapeutic use; Behçet's disease; Anti-TNFα; Neutrophilic dermatosis; Hidradenitis suppurativa; Tocilizumab; Ustekinumab; SAPHO; Pyoderma gangrenosum
• ISSN: 0049-0172; 1532-866X
• DOI: 10.1016/j.semarthrit.2023.152224

•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.
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