Department of Clinical Immunology and Internal Medicine, University of Lyon 1, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France.
Dermatology. 2023;239(2):177-187. doi: 10.1159/000526789. Epub 2022 Nov 9.
Atrophic papulosis (AP) is a rare obliterating vasculopathy characterized by specific skin lesions. The etiology and the pathophysiology of the disease remain unclear. The treatment is still empirical, while the malignant form of the disease is associated with a poor prognosis.
The underlying pathogenesis of AP includes three mechanisms with vasculopathy, coagulopathy, and endothelial dysfunction. Benign and malignant forms of AP are described. The benign form is confined to the skin. The pathognomonic skin lesions evolve over time and are large papules with an atrophic porcelain-white center and an erythematous rim. However, systemic involvement can occur months or years after the initial skin features. In this latter case, the associated mortality is very high with a mortality rate of over 65% in some series. Gastrointestinal involvement and central nervous system infarctions are the most frequent causes of death. Treatment is empirical with the use of antiplatelet therapy, anticoagulants, steroids, intravenous immunoglobulins, and immunosuppressive agents. Recent evidence shows that eculizumab, a complement inhibitor, is the most effective therapy in malignant AP with gastrointestinal involvement of the disease and should be combined with treprostinil to prevent relapse.
萎缩性丘疹病（AP）是一种罕见的闭塞性血管病，其特征为特定的皮肤损害。该病的病因和发病机制仍不清楚。目前的治疗方法仍然是经验性的，而该病的恶性形式与预后不良相关。
AP 的潜在发病机制包括血管病变、凝血障碍和内皮功能障碍这三种机制。本文描述了良性和恶性形式的 AP。良性形式局限于皮肤。特征性的皮肤损害随着时间的推移而演变，表现为大的丘疹，中心有萎缩性瓷白色，周围有红斑。然而，系统性受累可在最初的皮肤特征出现数月或数年后发生。在后一种情况下，死亡率非常高，某些系列的死亡率超过 65%。胃肠道受累和中枢神经系统梗死是最常见的死亡原因。治疗是经验性的，包括抗血小板治疗、抗凝剂、类固醇、静脉注射免疫球蛋白和免疫抑制剂。最近的证据表明，补体抑制剂依库珠单抗在伴有胃肠道受累的恶性 AP 中是最有效的治疗方法，应与曲前列尼尔联合使用以预防复发。
