Datasheet1_Malignant atrophic papulosis treated with eculizumab and hirudin: a fatal case report and literature review.pdf

BackgroundMalignant atrophic papulosis (MAP) is a rare obliterative vasculopathy whose etiology and pathophysiological mechanisms remain unknown, and the treatment is still empirical. It can involve multiple systems, especially the gastrointestinal tract and central nervous system, and has a poor prognosis.
Case presentationA 20-year-old Chinese male appeared to have Widespread atrophic papules and plaques, intermittent abdominal pain, recurrent bowel perforation, and psoas abscess. The clinical diagnosis of MAP was supported by skin biopsy. He was then treated with anticoagulants, antiplatelets, glucocorticoids, and immunosuppressants and started on eculizumab and hirudin after the first surgical interventions. Despite the aggressive immunosuppression, anticoagulant, antiplatelet, humanized monoclonal antibodies, and surgery therapy, he died five months after presentation.
ConclusionsMAP is an extremely rare obliterative vasculopathy manifesting as benign cutaneous involvement or potentially malignant systemic involvement. MAP patients who exhibit any abdominal symptoms should undergo laparoscopy and evaluation in time and start on eculizumab and treprostinil as soon as possible, as the combination of them is presently the most effective treatment option for gastrointestinal MAP and hopefully reduce mortality.

背景
恶性萎缩性丘疹病（Malignant atrophic papulosis, MAP）是一种罕见的闭塞性血管病，其病因及病理生理机制尚未明确，治疗手段仍以经验性为主。该病可累及多系统，尤以胃肠道与中枢神经系统为著，预后不佳。

病例报告
一名20岁中国男性患者，出现广泛性萎缩性丘疹及斑块、间歇性腹痛、反复肠穿孔及腰大肌脓肿。皮肤活检结果支持恶性萎缩性丘疹病（MAP）的临床诊断。患者先后接受抗凝剂、抗血小板药物、糖皮质激素及免疫抑制剂治疗，首次手术后启动依库珠单抗（eculizumab）与水蛭素（hirudin）治疗。尽管予以强化免疫抑制、抗凝、抗血小板、人源化单克隆抗体及手术治疗，患者仍于就诊后5个月死亡。

结论
恶性萎缩性丘疹病（MAP）是一种极为罕见的闭塞性血管病，临床表现可为良性皮肤受累或潜在致命性的全身受累。对于出现任何腹部症状的MAP患者，应及时行腹腔镜检查并进行病情评估，并尽快启动依库珠单抗（eculizumab）与曲前列尼尔（treprostinil）联合治疗，目前该联合方案是胃肠道MAP最有效的治疗选择，有望降低患者死亡率。