Unit of Dermatology, Department of Medical, Surgical and Neurological Science, University of Siena, Siena, Italy.
Department of Medical Biotechnologies, University of Siena, Siena, Italy.
Intern Emerg Med. 2023 Nov;18(8):2245-2252. doi: 10.1007/s11739-023-03410-9. Epub 2023 Oct 6.
Behçet's disease (BD) is a heterogeneous multifactorial autoinflammatory disease characterized by a plethora of clinical manifestations. Cutaneous lesions are considered hallmarks of the disease. However, their evolution over time and a thorough description are scarcely reported in non-endemic regions. The aim of this study was to detail BD skin manifestations and their evolution over time in Italy, as well as the dermatological prognostic impact of specific cutaneous features in long-standing disease. Data were collected in a double fashion, both retrospectively and prospectively, from the AutoInflammatory Disease Alliance (AIDA) international registry dedicated to BD, between January 2022 and December 2022. A total of 458 Italian patients were included. When assessing skin manifestations course, the constant or sporadic presence or absence of cutaneous involvement between onset and follow-up was considered. Oral ulcers (OU) (88.4%) and genital ulcers (GU) (52.6%), followed by skin involvement (53.7%) represented the most common presenting mucocutaneous manifestations at disease onset. Up to the time of enrolment into the AIDA registry, 411 (93.8%) patients had suffered from OU and 252 (57.9%) from GU; pseudofolliculitis (PF) accounted for the most common skin manifestation (170 patients, 37.1%), followed by erythema nodosum (EN) (102 patients, 22.3%), skin ulcers (9 patients, 2%) and pyoderma gangrenosum (4 patients, 0.9%). A prospective follow-up visit was reported in 261/458 patients; 24/148 (16.2%) subjects with skin involvement as early as BD onset maintained cutaneous lesions for the entire period of observation, while 120 (44.1%) patients suffered from sporadic skin involvement. Conversely, 94/113 (83.2%) with no skin involvement at disease onset did not develop skin lesions thereafter. At follow-up visits, cutaneous involvement was observed in 52 (20%) patients, with a statistically significant association between PF and constant skin involvement (p = 0.031). BD in Italy is characterized by a wide spectrum of clinical presentations and skin manifestations in line with what is described in endemic countries. Patients with skin disease at the onset are likely to present persistent cutaneous involvement thereafter; mucocutaneous lesions observed at the onset, especially PF, could represent a warning sign for future persistent skin involvement requiring closer dermatological care.
白塞病（BD）是一种异质性多因素自身炎症性疾病，其特征是临床表现多种多样。皮肤损伤被认为是该病的标志。然而，在非流行地区，其随时间的演变和详细描述很少被报道。本研究的目的是详细描述意大利 BD 的皮肤表现及其随时间的演变，以及在长期疾病中特定皮肤特征对皮肤病学预后的影响。数据以回顾性和前瞻性相结合的方式从专门针对 BD 的自身炎症性疾病联盟（AIDA）国际注册中心收集，时间为 2022 年 1 月至 2022 年 12 月。共纳入 458 名意大利患者。在评估皮肤表现过程时，考虑了发病和随访之间皮肤受累的持续或偶发存在或不存在。口腔溃疡（OU）（88.4%）和生殖器溃疡（GU）（52.6%），其次是皮肤受累（53.7%），是疾病发病时最常见的黏膜皮肤表现。截至 AIDA 登记处登记时，411 名（93.8%）患者患有 OU，252 名（57.9%）患者患有 GU；假性毛囊炎（PF）是最常见的皮肤表现（170 例，37.1%），其次是结节性红斑（EN）（102 例，22.3%）、皮肤溃疡（9 例，2%）和坏疽性脓皮病（4 例，0.9%）。261/458 名患者报告了前瞻性随访；148 名中有 24 名（16.2%）皮肤受累患者在 BD 发病时就出现了皮肤病变，整个观察期间都保持着皮肤病变，而 120 名（44.1%）患者患有偶发性皮肤受累。相反，113 名无皮肤受累的患者在发病时，此后没有出现皮肤病变。在随访时，52 名（20%）患者出现皮肤受累，PF 与持续皮肤受累之间存在统计学显著关联（p=0.031）。意大利的 BD 表现为广泛的临床表现和皮肤表现，与流行地区描述的相符。发病时患有皮肤病的患者此后更有可能出现持续性皮肤受累；发病时观察到的黏膜皮肤病变，尤其是 PF，可能是未来持续性皮肤受累需要更密切的皮肤科护理的预警信号。
