Ainechi Sanaz, Carlson John Andrew
Department of Pathology, Albany Medical College, Albany, NY.
Am J Dermatopathol. 2016 Feb;38(2):e22-6. doi: 10.1097/DAD.0000000000000358.
Neutrophilic dermatosis (ND) confined to postmastectomy lymphedema, localized Sweet syndrome, is a newly recognized disease. In this study, the authors describe a 44-year-old obese woman with chronic myelogenous leukemia in molecular remission on dasatinib therapy, who presented with a painful urticarial eruption limited to lipo-lymphedematous skin and accompanied by malaise, episodic fever, diarrhea, neutrophilia, and leukocytosis. Initially transient and migratory, the rash became fixed, papular, and vesicular and showed minimal response to corticosteroids. Biopsy demonstrated sparse perivascular and interstitial dermal neutrophilic infiltrates, without vasculitis or significant dermal edema. Aggregates of neutrophils were found within and surrounding lymphangiectases. Biopsy of a new onset papule 3 weeks later demonstrated papillary dermal edema, denser neutrophilic infiltrate, and vasculitis-like changes. These 2 histopathologic patterns of ND, early and late, resemble neutrophilic urticarial dermatitis (also known as neutrophilic dermatitis with systemic inflammation) and Sweet syndrome, respectively. Extensive workup did not reveal evidence of relapsed chronic myelogenous leukemia, infection, or a coexisting systemic inflammatory disease. Dasatinib was discontinued and the eruption gradually resolved over 2.5 months. Still in molecular remission (no detectable BCR-ABL gene fusion), dasatinib therapy was recommenced at 3-month follow-up. After 10 months, she complains of malaise and arthralgia, but no cutaneous symptoms. The evolution and slow resolution of this ND in lipo-lymphedematous skin implicate poor lymphatic clearance of factors, antigenic and/or toxic, involved in the pathogenesis of ND.
局限于乳房切除术后淋巴水肿的中性粒细胞性皮肤病(ND),即局限性Sweet综合征,是一种新发现的疾病。在本研究中,作者描述了一名44岁的肥胖女性,她患有慢性粒细胞白血病,正在接受达沙替尼治疗且处于分子缓解期,出现了仅限于脂肪性淋巴水肿皮肤的疼痛性荨麻疹样皮疹,并伴有不适、间歇性发热、腹泻、中性粒细胞增多和白细胞增多。皮疹最初短暂且游走,之后变为固定的丘疹和水疱,对皮质类固醇反应极小。活检显示血管周围和间质真皮有稀疏的中性粒细胞浸润,无血管炎或明显的真皮水肿。在淋巴管扩张内及周围发现了中性粒细胞聚集。3周后对新发丘疹进行活检,显示乳头真皮水肿、更密集的中性粒细胞浸润和血管炎样改变。ND的这两种早期和晚期组织病理学模式分别类似于中性粒细胞性荨麻疹性皮炎(也称为伴有全身炎症的中性粒细胞性皮炎)和Sweet综合征。全面检查未发现慢性粒细胞白血病复发、感染或并存的全身性炎症性疾病的证据。停用达沙替尼后,皮疹在2.5个月内逐渐消退。仍处于分子缓解期(未检测到BCR-ABL基因融合),在3个月随访时重新开始达沙替尼治疗。10个月后,她主诉不适和关节痛,但无皮肤症状。这种发生于脂肪性淋巴水肿皮肤的ND的演变和缓慢消退提示参与ND发病机制的抗原性和/或毒性因素的淋巴清除不良。