AI Article Synopsis
- This study examines the relationship between neutrophilic dermatosis (ND), hidradenitis suppurativa (HS), and Behçet's disease (BD), noting shared traits that suggest a common underlying cause.
- The research identified 20 patients (out of 1462 experiencing BD) with ND or HS, finding a relatively high occurrence of pyoderma gangrenosum (PG) among them, which highlights the unique clinical features and responses to treatment in these patients.
- Treatment options including colchicine, glucocorticoids, and various biologic therapies like anti-TNFα have shown promising results for managing refractory cases of ND and HS in the context of BD.
Article Abstract
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.
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| http://dx.doi.org/10.1016/j.semarthrit.2023.152224 | DOI Listing |
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