Pearson David R, Fujita Mayumi, High Whitney A
University of Colorado School of Medicine in Aurora, USA.
J Mol Biomark Diagn. 2017 May;8(3). doi: 10.4172/2155-9929.1000337. Epub 2017 Mar 2.
We describe two unique cases of fulminant mycosis fungoides with remarkably similar and aggressive clinical courses resulting in death. Both cases demonstrated ulcerated palmar and periorbital plaques and marked tissue eosinophilia, which was confirmed by T-cell receptor γ chain gene rearrangement studies to display identical monoclonality at temporally and anatomically distinct sites. Dense eosinophilic infiltrates on biopsy led to misdiagnosis of inflammatory dermatoses in both instances. While mycosis fungoides may be challenging to diagnose histologically, the presence of eosinophils in progressive disease may herald a poor prognosis and should not exclude the diagnosis.
我们描述了两例暴发性蕈样肉芽肿的独特病例，其临床病程极为相似且具有侵袭性，最终导致死亡。两例病例均表现为手掌和眶周溃疡斑块以及明显的组织嗜酸性粒细胞增多，经T细胞受体γ链基因重排研究证实，在时间和解剖位置不同的部位显示出相同的单克隆性。活检时密集的嗜酸性粒细胞浸润在两例中均导致炎症性皮肤病的误诊。虽然蕈样肉芽肿在组织学上可能难以诊断，但进行性疾病中嗜酸性粒细胞的存在可能预示预后不良，且不应排除该诊断。
