Chen Haonan, Li Chao, Ye Wenli, Ye Wei, Xu Hui, Jiang Qingwei, Huo Zhen, Zhao Xinyan, Li Hang
Department of Internal Medicine.
Department of Nephrology.
Medicine (Baltimore). 2020 Dec 4;99(49):e23492. doi: 10.1097/MD.0000000000023492.
Understanding the association between Henoch-Schönlein purpura (HSP) and malignancy is essential for early diagnosis and treatment of the potential lethal disease. To the best of our knowledge, there has been only one published case of HSP coexisting with oesophageal cancer. Here, we report another patient diagnosed with HSP and oesophageal squamous carcinoma simultaneously.
A 60-year-old Chinese male was referred to our hospital because of intermittent abdominal pain, abdominal distension, melena, lower extremities purpura. Positive laboratory values included pancytopenia, microscopic hematuria, nephrotic proteinuria, hematochezia, hypoalbuminemia, hyperlipidaemia, hypocomplementemia, and increased levels of hepatobiliary enzymes and immunoglobulin (Ig) A. Gastrocolonoscopy showed multiple erosion lesion on descending duodenum, terminal ileum, and ileal flap. Biopsy of these lesions suggested non-specific inflammation.
HSP (IIIb type) was diagnosed based on renal pathology examination in accordance with the International Study of Kidney Disease in Children (ISKDC) classification. Liver biopsy confirmed the diagnosis of nodular cirrhosis (Ishak 5). Gastroscopy unintentionally revealed three oesophagus lesions. Pathology study suggested intermediate differentiated squamous cell carcinoma (cTNM IB).
Before admission, he was administered intravenous Ig 10 g once daily(qd) for 10 days, methylprednisolone 40 mg qd for a week, followed by prednisolone 50 mg qd for almost 8 weeks. Endoscopic submucosal dissection (ESD) was performed to remove all lesions with negative margin after prednisolone was tapered (5 mg per week until 10 mg qd).
Despite prednisone being tapered to 2.5 mg qd within 2 months, complete remission of HSP and esophageal malignancy was achieved after the resection of the esophagus lesions during 12 months follow-up.
We report a rare case of oesophageal squamous cell carcinoma initially presented as HSP. This case suggests the importance of evaluating adult patients with HSP for an underlying malignancy.
了解过敏性紫癜（HSP）与恶性肿瘤之间的关联对于这种潜在致命疾病的早期诊断和治疗至关重要。据我们所知，仅有一例HSP与食管癌共存的病例发表。在此，我们报告另一例同时诊断为HSP和食管鳞状细胞癌的患者。
一名60岁的中国男性因间歇性腹痛、腹胀、黑便、下肢紫癜被转诊至我院。实验室检查阳性结果包括全血细胞减少、镜下血尿、肾病性蛋白尿、便血、低白蛋白血症、高脂血症、补体降低以及肝胆酶和免疫球蛋白（Ig）A水平升高。胃肠镜检查显示降部十二指肠、回肠末端和回盲瓣有多处糜烂性病变。这些病变的活检提示非特异性炎症。
根据儿童肾脏病国际研究（ISKDC）分类，基于肾脏病理检查诊断为HSP（IIIb型）。肝活检确诊为结节性肝硬化（Ishak 5级）。胃镜意外发现三处食管病变。病理研究提示为中分化鳞状细胞癌（cTNM IB期）。
入院前，他接受了静脉注射Ig 10g每日一次（qd）共10天，甲泼尼龙40mg qd一周，随后泼尼松龙50mg qd持续近8周。在泼尼松龙逐渐减量（每周5mg直至10mg qd）后，进行了内镜下黏膜下剥离术（ESD）以切除所有切缘阴性的病变。
尽管泼尼松在2个月内逐渐减至2.5mg qd，但在12个月的随访期间，食管病变切除后HSP和食管恶性肿瘤均实现了完全缓解。
我们报告了一例罕见的最初表现为HSP的食管鳞状细胞癌病例。该病例提示评估成年HSP患者是否存在潜在恶性肿瘤的重要性。
