Sayed Christopher J, El-Behaedi Salma E, Osborn Lindsay P, Googe Paul B, Blum Franklin R
Drs. Sayed and Osborn are with the Department of Dermatology at UNC Chapel Hill School of Medicine in Chapel Hill, North Carolina. Ms. El-Behaedi and Mr. Blum are also with the Department of Dermatology at UNC Chapel Hill School of Medicine in Chapel Hill, North Carolina.
Dr. Googe is with the UNC Chapel Hill School of Medicine, UNC Department of Pathology and Laboratory Medicine, and the UNC Lineberger Comprehensive Cancer Center in Chapel Hill, North Carolina.
J Clin Aesthet Dermatol. 2023 Apr;16(4):28-31.
No known studies have attempted to describe the pathophysiological relationship between patients who develop both porokeratosis and hidradenitis suppurativa (HS). The purpose of this report is to present possible immunological mechanisms that predispose patients to developing both porokeratosis and HS.
In this case series, patients were identified during routine clinical encounters and data was extracted from the electronic medical record from October 2010 until April 2021. This study is a single center case series including patients from the department of dermatology at the UNC School of Medicine in Chapel Hill, North Carolina. Patients were selected via digital chart review if they had simultaneous diagnoses of disseminated porokeratosis and HS. Two eligible patients were identified as actively receiving care. One patient is a Black female and the other a White male. No primary study outcomes were planned. This investigation utilized chart review to identify disease time course, which was subsequently used to elucidate study outcomes.
Patient A is a 54-year-old Black female and Patient B is a 65-year-old White male. Both patients developed porokeratosis after multiple years of living with HS. Immunosuppression with adalimumab, corticosteroids, or other medications did not clearly precede porokeratosis development in either patient.
Limitations include that this study was conducted at a single center and prevalence of patients with concomitance of both conditions is low.
In patients who demonstrate simultaneous HS and porokeratosis, activation of the innate immune system and associated IL-1 production may lead to autoinflammation and a phenotype of hyperkeratinization. Mutations in genes such as mevalonate kinase may predispose subjects to the development of porokeratoses and HS.
尚无已知研究试图描述同时发生汗孔角化症和化脓性汗腺炎（HS）的患者之间的病理生理关系。本报告的目的是提出可能使患者易患汗孔角化症和HS的免疫机制。
在这个病例系列中，患者是在常规临床诊疗过程中被识别出来的，数据从2010年10月至2021年4月的电子病历中提取。本研究是一个单中心病例系列，包括来自北卡罗来纳州教堂山分校北卡罗来纳大学医学院皮肤科的患者。如果患者同时被诊断为播散性汗孔角化症和HS，则通过数字病历审查进行选择。两名符合条件的患者被确定为正在接受治疗。一名患者是黑人女性，另一名是白人男性。未计划主要研究结果。本调查利用病历审查来确定疾病病程，随后用于阐明研究结果。
患者A是一名54岁的黑人女性，患者B是一名65岁的白人男性。两名患者在患有HS多年后都出现了汗孔角化症。在任何一名患者中，使用阿达木单抗、皮质类固醇或其他药物进行免疫抑制均未明显先于汗孔角化症的发生。
局限性包括本研究是在单一中心进行的，两种疾病同时存在的患者患病率较低。
在同时患有HS和汗孔角化症的患者中，先天免疫系统的激活和相关白细胞介素-1的产生可能导致自身炎症和角化过度的表型。甲羟戊酸激酶等基因的突变可能使个体易患汗孔角化症和HS。
