An ultrastructual examionation of a blistering lesion of mycosis fungoides bullosa
An ultrastructual examionation of a blistering lesion of mycosis fungoides bullosa
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大疱蕈样肉芽肿起泡病灶的超微结构检查
DOI：
10.1111/j.1365-2133.2011.10323.x
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发表时间：
2011
期刊：
影响因子：
--
通讯作者：
et. al
中科院分区：
文献类型：
--
作者：
Ueda C.;Makino T.;et. al
蕈样真菌病（Mycosis fungoides， MF）是最常见的皮肤t细胞淋巴瘤，其临床表现多种多样。在众多的临床变型中，以囊泡状表现的MF （MF bullosa， MFB）极为罕见。MF患者出现MFB被认为预后不良。迄今为止，仅报道了12例MF的囊泡性病变。2,3这篇报告描述了MFB的起泡病变的电镜观察。49岁男性，47岁时被诊断为MF，表现为全身瘙痒性红斑疹。他接受了补骨脂素加紫外线疗法和局部应用皮质类固醇的治疗。他在50岁时因红皮病和高烧住院。他有明显的颈部和腋窝淋巴结病变。肝脏和脾脏未触及。他接受了四个周期的化疗，包括环磷酰胺、盐酸阿霉素、硫酸长春新碱和强的松（CHOP）。在CHOP治疗期间，患者右臂红斑病变上出现了几个直径达10mm的刚性囊泡（图1a）。他没有虫咬、病毒感染、其他药物、烧伤或接触性过敏史。囊泡的组织学结果显示表皮下水疱和真皮上部肿瘤细胞、中性粒细胞和嗜酸性粒细胞的带状浸润（图1b， c）。免疫荧光检查未见表皮免疫球蛋白或补体沉积。血检嗜酸性粒细胞数目在正常范围内。排除了CHOP引起的药疹的可能性，因为尽管继续CHOP治疗，但病变未经治疗就消失了。这些结果对MFB的水泡性病变具有诊断价值。接下来我们进行了超微结构检查。电镜显示大量水泡和胶原束变性。虽然基底膜看起来完好无损，但基底角质形成细胞也受到轻微损伤（图2a）。非典型淋巴细胞在靠近表皮下水泡处的细胞核处有一个深切口（图2b）。虽然MFB的病变最终消失了，但是
MADAM, Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma, and its clinical manifestations are diverse. Among the numerous clinical variants, the vesiculobullous manifestation of MF, named MF bullosa (MFB), is extremely rare. The appearance of MFB in patients with MF has been suggested to be a poor prognosis. 1 Only 12 cases of vesiculobullous lesions in MF have been reported to date. 2, 3 This report describes an electron microscopic observation of a blistering lesion of MFB.A 49-year-old man, who had been diagnosed with MF at 47 years of age, presented with generalized pruritic erythematous eruptions. He had received treatment with psoralen plus ultraviolet A therapy and a topical application of corticosteroids. He was hospitalized at 50 years of age, because of erythroderma and high fever. He had noticeable cervical and axillary lymphadenopathy. The liver and the spleen were not palpable. He received four cycles of chemotherapy, including cyclophosphamide, doxorubicin hydrochloride, vincristine sulphate and prednisone (CHOP). Several rigid vesicles measuring up to 10 mm in diameter developed on the erythematous lesions of his right arm during CHOP therapy (Fig. 1a). He had no history of insect bites, viral infections, other medications, burns or contact allergy. The histological findings from a vesicle showed a subepidermal blister and a band-like infiltration of tumour cells, neutrophils and eosinophils in the upper dermis (Fig. 1b, c). No deposition of either immunoglobulin or complement was observed in the epidermis by immunofluorescent examination. The number of eosinophils was within the normal limit in the blood tests. The possibility that the lesions were a drug eruption caused by CHOP was excluded, because the lesions disappeared without treatment in spite of the continuation of CHOP therapy. These findings were diagnostic for the blistering lesions of MFB. 1 We next performed an ultrastructural examination. 4 Electron microscopy revealed numerous blisters and degenerated collagen bundles. Although the basal lamina appeared to be intact, the basal keratinocytes were also slightly injured (Fig. 2a). Atypical lymphocytes that had a deep notch in the nuclei were observed close to the blisters in the subepidermis (Fig. 2b). Although the lesions of MFB eventually disappeared, the