Department of Dermatology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nuremberg (FAU), Erlangen, Germany.
Department of Dermatology, Venerology and Allergology, University Hospital Essen, Essen, Germany.
Dermatology. 2022;238(1):148-155. doi: 10.1159/000514687. Epub 2021 Apr 7.
Necrobiosis lipoidica (NL) is a rare granulomatous disorder of unknown aetiology. Randomized controlled studies are not available due to it being an orphan disease.
We evaluated patients in 2 dermatological centres to cluster data about epidemiology, the therapeutic approaches for NL, and their efficacy.
Comorbidity and the efficacy of the applied treatment was assessed for 98 patients.
We identified 54% of patients with concomitant diabetes and 19% with thyroidal disorders. Topical steroids (85.7%) were predominantly used followed by calcineurin inhibitors (31%) and phototherapy (41.8%). Systemically, fumaric acid esters were more frequently applied (26.8%) than steroids (24.4%) and dapsone (24.4%). Steroids, compression therapy, calcineurin inhibitors, phototherapy, fumaric acid esters, and dapsone showed remarkable efficacy.
Therapeutic options were chosen individually in accordance with the severity of NL and presence of ulceration. Topical calcineurin inhibitors, systemic application of fumaric acid esters, and dapsone represent effective alternatives to the use of steroids.
脂性渐进性坏死(NL)是一种病因不明的罕见肉芽肿性疾病。由于该病属于罕见病,因此尚无随机对照研究。
我们在 2 个皮肤科中心评估了患者,以对 NL 的流行病学、治疗方法及其疗效进行聚类数据分析。
我们评估了 98 例患者的合并症和应用治疗的疗效。
我们发现 54%的患者伴有糖尿病,19%的患者伴有甲状腺疾病。局部应用皮质类固醇(85.7%)最为常见,其次是钙调磷酸酶抑制剂(31%)和光疗(41.8%)。系统应用富马酸酯(26.8%)比皮质类固醇(24.4%)和氨苯砜(24.4%)更为常见。皮质类固醇、加压治疗、钙调磷酸酶抑制剂、光疗、富马酸酯和氨苯砜均显示出显著的疗效。
根据 NL 的严重程度和溃疡的存在,选择了个体化的治疗方案。局部钙调磷酸酶抑制剂、全身应用富马酸酯和氨苯砜是替代皮质类固醇的有效方法。