PDF(Pubmed)
Abstract：
Sweet\'s syndrome (SS), or acute febrile neutrophilic dermatosis, characteristically presents with fever, dermal neutrophilic infiltrates, and neutrophilia. It typically manifests as tender erythematous plaques; however, various variants are documented, including bullous. Malignancy-associated Sweet\'s syndrome (MASS) can present as a paraneoplastic syndrome in those with established cancers or with undiagnosed malignancies. We present a 72-year-old male with a three-day history of a progressive bullous, erythematous papular rash starting on his right forearm and spreading to his extremities, trunk, palms, and soles. It was mildly pruritic but nontender. He had no recent febrile illnesses. On examination, the rash was violaceous with tense bullae overlying edematous targetoid papules coalescing into plaques. Histopathologic analysis of punch biopsies from his abdomen and thigh demonstrated dense inflammatory infiltrates of neutrophils, eosinophils, histiocytes, and lymphocytes, suggestive of neutrophilic dermatosis, or Sweet\'s syndrome. He was treated with prednisone 1 mg/kg with improvement in his cutaneous symptoms, and a malignancy workup was initiated. Blood work showed elevated free kappa, lambda light chains, lactate dehydrogenase (LDH), and C-reactive protein (CRP) levels. A positron emission tomography (PET) scan revealed lesions in the esophagus and kidney. He was referred to Heme/Onc, GI, and Urology. He was diagnosed with esophageal adenocarcinoma stage IIb and a renal mass. He has since completed neoadjuvant chemotherapy and radiation, is s/p robotic Ivor-Lewis esophagectomy with no evidence of residual carcinoma on pathology, and is undergoing surveillance with imaging every three months for his renal mass. This case highlights the importance of rapid identification of MASS and the impact dermatologists can make in getting these patients the potentially lifesaving care they need.
摘要：
甜综合征（SS），或急性发热性嗜中性粒细胞性皮肤病，典型地表现为发烧，真皮嗜中性粒细胞浸润，和嗜中性粒细胞增多症.它通常表现为嫩红斑；然而，记录了各种变体，包括bullous.恶性肿瘤相关的Sweet’s综合征（MASS）可作为一种副肿瘤综合征出现在已确诊的癌症或未确诊的恶性肿瘤患者中。我们介绍了一名72岁的男性，他有三天的进行性大疱病史，红斑丘疹从他的右前臂开始并扩散到他的四肢，树干,手掌,和鞋底。轻度瘙痒，但不嫩。他最近没有发热性疾病。在检查中，皮疹为紫罗兰色，紧张的大疱覆盖着水肿的目标状丘疹，并合并成斑块。从他的腹部和大腿穿刺活检的组织病理学分析显示中性粒细胞的密集炎症浸润，嗜酸性粒细胞,组织细胞,和淋巴细胞，提示嗜中性皮肤病，或甜蜜综合症。他接受泼尼松1mg/kg治疗，皮肤症状得到改善，并开始了恶性肿瘤检查。血液检查显示游离Kappa升高,λ轻链，乳酸脱氢酶（LDH），和C反应蛋白（CRP）水平。正电子发射断层扫描（PET）扫描显示食管和肾脏有病变。他被转介给Heme/Onc,GI,和泌尿科。他被诊断为食管腺癌IIb期和肾脏肿块。此后他完成了新辅助化疗和放疗，是s/p机器人Ivor-Lewis食管切除术，病理学上没有残留癌的证据，并且每三个月对他的肾脏肿块进行成像监测。这个案例强调了快速识别MASS的重要性，以及皮肤科医生可以为这些患者提供所需的潜在救生护理。
