Rogers Meredith C, Ash Mark, Hernandez Amanda, Hosler Gregory A
Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Empire Dermatology, Syracuse, New York, USA.
J Cutan Pathol. 2025 Apr;52(4):272-277. doi: 10.1111/cup.14774. Epub 2025 Jan 3.
The term autoinflammatory keratinization diseases (AIKDs) was recently proposed as a unifying concept for diseases characterized by inflammation in the epidermis and upper dermis which leads to hyperkeratosis, caused by genetic perturbations of the innate immune system. We present a case of a patient with hidradenitis suppurativa and porokeratosis, two AIKDs, followed by a review of these conditions as well as other AIKDs. This case was distinguished by hypertrophic porokeratoses involving cystic hair follicles, showing histopathologic features of both conditions within single biopsy specimens. The patient's course was additionally complicated by SCC arising within a porokeratosis. Our case demonstrates a rare overlap of two AIKDs, occurring not only within the same patient but also within the same lesions.
自身炎症性角化病（AIKDs）这一术语最近被提出，作为一种统一的概念，用于描述由先天性免疫系统的基因扰动导致的、以表皮和真皮上层炎症并进而引起角化过度为特征的疾病。我们报告一例患有化脓性汗腺炎和汗孔角化症这两种AIKDs的患者病例，随后对这些病症以及其他AIKDs进行综述。该病例的特征是肥厚性汗孔角化症累及囊性毛囊，在单个活检标本中显示出两种病症的组织病理学特征。此外，患者病程因汗孔角化症内发生鳞状细胞癌而复杂化。我们的病例展示了两种AIKDs罕见的重叠情况，不仅发生在同一患者身上，还出现在同一病变内。
