Wang Michael Z, Camilleri Michael J, Guo Ruifeng, Wieland Carilyn N
Department of Dermatology, Mayo Clinic, Rochester, Minnesota.
Division of Anatomic Pathology, Mayo Clinic, Rochester, Minnesota.
J Cutan Pathol. 2017 Apr;44(4):367-372. doi: 10.1111/cup.12873. Epub 2017 Jan 19.
Amicrobial pustulosis of the folds (APF) is a rare disease characterized by aseptic pustular lesions involving cutaneous folds, typically occurring in the context of an autoimmune disorder. We present 4 patients with APF, focusing on clinical and histopathologic characteristics to improve the recognition of this entity. All 4 patients had intertriginous and extra-intertriginous involvement. Common histopathologic features of skin biopsies in these patients were intracorneal, subcorneal, intraepidermal, perivascular, perifollicular and interstitial neutrophilic inflammation. Pustules overlying adnexal ostium and papillary dermal edema were consistently observed. The pustules were negative for microorganisms on stain testing. In these cases, associated conditions were undifferentiated connective tissue disease, systemic lupus erythematosus, antiphospholipid syndrome, Crohn disease and Hashimoto thyroiditis. The aforementioned clinical and histopathologic characteristics of patients with suspected or diagnosed connective tissue disorders should lead to suspicion for APF.
褶皱部无菌性脓疱病（APF）是一种罕见疾病，其特征为累及皮肤褶皱的无菌性脓疱性病变，通常发生于自身免疫性疾病背景下。我们报告4例APF患者，重点关注临床和组织病理学特征以提高对该疾病的认识。所有4例患者均有擦烂处及非擦烂处受累。这些患者皮肤活检的常见组织病理学特征为角质层内、角质层下、表皮内、血管周围、毛囊周围及间质中性粒细胞炎症。始终观察到覆盖附属器开口处的脓疱及乳头真皮水肿。染色检测显示脓疱内无微生物。在这些病例中，相关疾病有未分化结缔组织病、系统性红斑狼疮、抗磷脂综合征、克罗恩病及桥本甲状腺炎。疑似或诊断为结缔组织病患者的上述临床和组织病理学特征应引起对APF的怀疑。
