Snow J L, Muller S A
Department of Dermatology, Mayo Clinic, Rochester, MN 55905, USA.
Semin Dermatol. 1995 Jun;14(2):99-105. doi: 10.1016/s1085-5629(05)80004-5.
Malignant atrophic papulosis is a rare disorder characterized by pathognomonic cutaneous lesions that have been associated with multiple infarctive thrombotic lesions of other viscera, most notably the gastrointestinal tract and the central nervous system. Systemic involvement may develop from weeks to years after the onset of the characteristic cutaneous lesions or, rarely, may precede the cutaneous lesions. However, the existence of patients with a prolonged, purely cutaneous variant of this disease has been increasingly appreciated, and this brings into question the appropriateness of applying the term "malignant" to all patients who have the peculiar characteristic cutaneous lesions of malignant atrophic papulosis. Despite half a century of sporadic investigation, the precise cause of this disease remains unknown, and accurate classification of this entity as a primary vasculopathy or primary coagulopathy has not been possible. Unfortunately, no effective therapy exists for those patients in whom systemic involvement develops.
恶性萎缩性丘疹病是一种罕见的疾病，其特征为具有诊断意义的皮肤病变，这些病变与其他内脏器官的多发性梗死性血栓形成病变有关，最显著的是胃肠道和中枢神经系统。系统性受累可能在特征性皮肤病变出现后的数周或数年发生，或者很少情况下可能先于皮肤病变出现。然而，越来越多的人认识到存在这种疾病的长期单纯皮肤变异型患者，这使得将“恶性”一词应用于所有具有恶性萎缩性丘疹病特征性皮肤病变的患者是否合适受到质疑。尽管经过了半个世纪的零星研究，这种疾病的确切病因仍然未知，并且将该实体准确分类为原发性血管病或原发性凝血病是不可能的。不幸的是，对于那些出现系统性受累的患者，不存在有效的治疗方法。
