Magro Cynthia M, Toledo-Garcia Aixa, Pala Ozlem, Momtahen Shabnam, Shapiro Lee
Weill Cornell College of Cornell University.
Dermatol Online J. 2015 Sep 17;21(9):13030/qt7dk8q7n1.
In 2012, a nephrologist reported the development of a multiorgan thrombotic syndromic complex resembling thrombotic thrombocytopenic purpura (TTP) in patients who were abusing long acting oxymorphone hydrochloride; all patients had hemolytic anemia and thrombocytopenia.
Herein, we report another case involving a 31-year-old woman who self intravenously administered dissolved oral oxymorphone resulting in thrombotic sequelae resembling Degos disease.
Formalin-fixed and paraffin embedded skin biopsies were prepared according to standard protocols for H&E and immunohistochemistry.
The clinical presentation and biopsy findings were held to be indicative of Degos disease/malignant atrophic papulosis (MAP) but with unusual clinical features including renal failure and severe respiratory insufficiency. Given the efficacy of eculizumab in the treatment of the acute thrombotic phase of Degos disease/MAP, the patient received this drug, resulting in rapid resolution of signs and symptoms associated with her multiorgan failure. Although she developed recurrent cutaneous ulcers despite complete complement inhibition with eculizumab., her other extracutaneous manifestations did not recur. The patient's pre and post eculizumab skin biopsies showed a striking pauci-inflammatory thrombogenic vasculopathy associated with marked endothelial cell injury along with deposits of C3d and C4d within the cutaneous vasculature; the C5b-9 deposits were limited to the pre-eculizumab biopsy. We discovered that her syndromic complex was a self-inflicted one related to the localized administration of dissolved oxymorphone.
Our patient's biopsy along with the rapid response to eculizumab indicates that this distinct thrombotic microangiopathy is another complement mediated thrombotic microangiopathy syndrome. Opioid thrombotic microangiopathy has a varied clinical presentation and can mimic other catastrophic microangiopathy syndromes, all of which have in common a responsiveness to complement inhibition.
2012年，一名肾病学家报告称，滥用长效盐酸羟吗啡酮的患者出现了一种类似于血栓性血小板减少性紫癜（TTP）的多器官血栓形成综合征；所有患者均有溶血性贫血和血小板减少症。
在此，我们报告另一例病例，一名31岁女性自行静脉注射溶解后的口服羟吗啡酮，导致出现类似于德戈斯病的血栓后遗症。
按照标准方案制备福尔马林固定、石蜡包埋的皮肤活检标本，用于苏木精-伊红染色（H&E）和免疫组织化学检查。
临床表现和活检结果被认为提示德戈斯病/恶性萎缩性丘疹病（MAP），但具有不寻常的临床特征，包括肾衰竭和严重呼吸功能不全。鉴于依库珠单抗在治疗德戈斯病/MAP急性血栓形成期的疗效，该患者接受了此药治疗，导致与多器官功能衰竭相关的体征和症状迅速缓解。尽管在使用依库珠单抗完全抑制补体后她仍出现复发性皮肤溃疡，但她的其他皮肤外表现未复发。该患者在使用依库珠单抗前后的皮肤活检显示，存在一种显著的少炎性血栓形成性血管病变，伴有明显的内皮细胞损伤，以及皮肤血管内C3d和C4d沉积；C5b-9沉积仅限于使用依库珠单抗前的活检标本。我们发现她的综合征是由局部注射溶解后的羟吗啡酮导致的自伤性综合征。
我们患者的活检结果以及对依库珠单抗的快速反应表明，这种独特的血栓性微血管病是另一种补体介导的血栓性微血管病综合征。阿片类药物所致血栓性微血管病临床表现多样，可模仿其他灾难性微血管病综合征，所有这些综合征的共同特点是对补体抑制有反应。
