Michelerio Andrea, Vassallo Camilla, Fiandrino Giacomo, Tomasini Carlo Francesco
Dermatology Clinic, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy.
Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, University of Pavia, 27100 Pavia, Italy.
Dermatopathology (Basel). 2021 Sep 23;8(4):450-462. doi: 10.3390/dermatopathology8040048.
Erosive pustular dermatosis of the scalp (EPDS) is an uncommon, pustular, idiopathic disorder typically occurring on the scalp of the elderly, whose diagnosis requires close clinicopathologic correlations. Recently, the primary histopathologic characteristic of EPDS has been identified in some biopsies from hair-bearing scalp lesions as a sterile, vesiculo-pustule involving the infundibulum of hair follicles. To further delineate the clinicopathologic spectrum of the disease, we led a retrospective study of 50 patients (36 males and 14 females) with a diagnosis of EPDS between 2011 and 2021, reviewing clinical and histopathological data. Androgenetic alopecia was present in 32 patients. Triggering factors were present in 21 patients. The vertex was the most common location; one patient also had leg involvement. Two cases were familial. Disease presentation varied markedly from tiny, erosive, scaly lesions to crusted and hemorrhagic plaques, mimicking pustular pyoderma gangrenosum (PPG). Biopsies of patients with severe androgenetic or total baldness produced specimens showing nonspecific pathologic changes (39/50), while in 11 patients with a hair-bearing scalp histopathologic examination, changes were specific. The clinicopathologic similarities between EPDS and PPG suggest that EPDS should be included in the spectrum of autoinflammatory dermatoses. Clinicians could consider the possibility of associated disorders rather than managing EPDS as a sui generis skin disorder.
头皮糜烂性脓疱病（EPDS）是一种罕见的脓疱性特发性疾病，通常发生在老年人头皮上，其诊断需要密切的临床病理关联。最近，在一些来自有毛发头皮病变的活检中，EPDS的主要组织病理学特征被确定为一种累及毛囊漏斗部的无菌性水疱脓疱。为了进一步描述该疾病的临床病理范围，我们对2011年至2021年间诊断为EPDS的50例患者（36例男性和14例女性）进行了一项回顾性研究，回顾了临床和组织病理学数据。32例患者存在雄激素性脱发。21例患者存在诱发因素。头顶是最常见的发病部位；1例患者腿部也有累及。2例为家族性。疾病表现差异很大，从微小的糜烂性鳞屑性病变到结痂和出血性斑块，类似脓疱型坏疽性脓皮病（PPG）。严重雄激素性脱发或全秃患者的活检标本显示非特异性病理改变（39/50），而11例有毛发头皮组织病理学检查的患者，其改变具有特异性。EPDS与PPG之间的临床病理相似性表明，EPDS应纳入自身炎症性皮肤病的范畴。临床医生应考虑相关疾病的可能性，而不是将EPDS作为一种独特的皮肤疾病来处理。
