Born S, Gaber G, Willgeroth K, Wagner U, Haneke E, Marsch W C
Department of Dermatology, Martin Luther-Universität Halle-Wittenberg, Ernst-Kromayer- Str. 5/6, D-06097, Halle(Saale), Germany.
Eur J Dermatol. 1999 Oct-Nov;9(7):569-73.
This paper presents the case of a 65 year-old woman suffering from recurrent oral aphthoid ulcers which rapidly evolved towards hyperplastic and ulcerated lesions over the entire floor of the mouth. The initial lesions were interpreted as non-specific aphthoid ulcers. Later, a tentative diagnosis of necrotising stomatitis with secondary reactive proliferating epithelial hyperplasia was made. The clinical symptoms and the immuno-phenotyping of lymphocytes circulating in the peripheral blood suggested the diagnosis of CD30-positive large cell anaplastic lymphoma. The biopsy showed only a pseudoepitheliomatous hyperplasia, reactive infiltrates and no lymphoma cells. The disease ran a fulminant course leading to death within 4 weeks due to acute gastro-intestinal bleeding. Autopsy revealed infiltrates of CD30+ large cell anaplastic lymphoma in a submandibular lymph node, in a thrombus stenosing the right subclavian vein, in the spleen, the anterior and posterior gastric wall as well as in the depth of the tumour on the floor of the mouth. The clinical and histopathological spectrum of CD30+ large cell anaplastic lymphoma is considerably variable. The particular feature of pseudoepitheliomatous hyperplasia has been reported especially in CD30+ anaplastic large cell lymphomas. An early correct diagnosis is rendered difficult in insufficient biopsy size, becauses this type of lymphoma often simulates other inflammatory or neoplastic skin diseases. Thus, with a necrotising and hyperplastic gingivostomatitis, the diagnosis of a CD30+ anaplastic large cell lymphoma should be considered.
本文介绍了一位65岁女性的病例,该患者患有复发性口腔口疮样溃疡,这些溃疡迅速发展为整个口腔底部的增生性和溃疡性病变。最初的病变被诊断为非特异性口疮样溃疡。后来,初步诊断为坏死性口炎伴继发性反应性增生性上皮增生。临床症状和外周血中循环淋巴细胞的免疫表型提示诊断为CD30阳性大细胞间变性淋巴瘤。活检仅显示假上皮瘤样增生、反应性浸润,未见淋巴瘤细胞。疾病呈暴发性经过,因急性胃肠道出血在4周内死亡。尸检显示在颌下淋巴结、狭窄右锁骨下静脉的血栓、脾脏、胃前壁和后壁以及口腔底部肿瘤深处有CD30 +大细胞间变性淋巴瘤浸润。CD30 +大细胞间变性淋巴瘤的临床和组织病理学表现差异很大。假上皮瘤样增生的特殊特征尤其在CD30 +间变性大细胞淋巴瘤中有所报道。活检样本不足时难以早期做出正确诊断,因为这种类型的淋巴瘤常模拟其他炎症性或肿瘤性皮肤病。因此,对于坏死性和增生性龈口炎,应考虑诊断为CD30 +间变性大细胞淋巴瘤。