Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
Center for Clinical Epidemiology and Clinical Statistics, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
Dermatology. 2023;239(2):248-254. doi: 10.1159/000528064. Epub 2023 Jan 18.
Adult-onset immunodeficiency (AOID) due to interferon-gamma autoantibody is a rare, acquired immunodeficiency disease. Reactive neutrophilic dermatoses (RND), predominantly Sweet syndrome (SS), and generalized pustular eruption have been reported repeatedly.
The aims of this study were to describe the cutaneous manifestations in AOID patients and determine the incidence of RND and associated factors using a larger population size than have been previously reported.
A retrospective chart review of all confirmed AOID cases in Chiang Mai University Hospital from January 2006 to June 2020 was conducted. The demographics and characteristics of RND including type, onset, and laboratory information in every episode of cutaneous manifestations were collected. Generalized estimating equations of binary logistic regression were used to determine the indicators of RND.
A total of 146 patients with confirmed AOID were identified. Of these, 57 cases (39%) developed at least one episode of RND. Thirteen cases (23%) of the patients experienced RND twice during the follow-up period. All recurrence of RND displayed the same cutaneous phenotype, with the exception of 2 cases who had both SS and generalized pustular eruption. Finally, 49 episodes of SS and 22 episodes of generalized pustular eruption were included in the analysis. All patients with RND had concomitant active opportunistic infections, of which most were non-tuberculous mycobacterium (NTM) infection. NTM infection (prevalence odds ratio [POR] 2.87), lymphadenopathy (POR 3.30) as well as lower serum alkaline phosphatase (ALP) level (POR 0.71 for every 100-unit increment in ALP) were found to be significantly associated with RND occurrence.
39% of our AOID patients experienced RND once during the course of the disease. Notable factors associated with RND occurrence were concomitant NTM infection, lymphadenopathy, and lower level of ALP.
成人发病的免疫缺陷（AOID）是一种由干扰素-γ自身抗体引起的罕见获得性免疫缺陷病。已反复报道过反应性中性粒细胞皮肤病（RND），主要是Sweet 综合征（SS）和全身性脓疱性发疹。
本研究旨在描述 AOID 患者的皮肤表现，并使用比以往报道更大的人群规模来确定 RND 的发生率和相关因素。
对 2006 年 1 月至 2020 年 6 月期间在清迈大学医院确诊的所有 AOID 病例进行回顾性病历审查。收集了每一次皮肤表现发作时 RND 的类型、发病时间和实验室信息等特征。使用二元逻辑回归的广义估计方程来确定 RND 的指标。
共确定了 146 例确诊的 AOID 患者。其中，57 例（39%）至少发生了一次 RND。在随访期间，有 13 例（23%）患者发生了两次 RND。所有 RND 的复发均表现出相同的皮肤表型，除了 2 例同时患有 SS 和全身性脓疱性发疹。最终，纳入分析的 SS 有 49 例，全身性脓疱性发疹有 22 例。所有 RND 患者均伴有活动性机会性感染，其中大多数为非结核分枝杆菌（NTM）感染。NTM 感染（患病率比 [POR] 2.87）、淋巴结病（POR 3.30）以及血清碱性磷酸酶（ALP）水平较低（ALP 每增加 100 单位，POR 为 0.71）与 RND 的发生显著相关。
我们的 AOID 患者中有 39%在疾病过程中发生过 RND。与 RND 发生相关的显著因素是伴有 NTM 感染、淋巴结病和较低的 ALP 水平。
