Lee Yin Yin, Bee Ping Chong, Lee Chew Kek, Naiker Manimalar, Ismail Rokiah
Department of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
Ann Dermatol. 2011 Dec;23(Suppl 3):S390-2. doi: 10.5021/ad.2011.23.S3.S390. Epub 2011 Dec 27.
Bullous pemphigoid (BP) has a recognized association with solid organ tumors, but is relatively rare in hematological malignancies. We report a 67-year-old male who developed BP after being diagnosed with myelodysplastic syndrome and refractory anemia with excess of blast (RAEB). Skin biopsy elucidated sub-epidermal bulla using direct immunofluorescence, revealing linear C3 and IgG deposits along the basement membrane. His BP was recalcitrant to the conventional treatment and only responded to a combination of high dose oral prednisolone and azathioprine. The relative refractory nature of his condition and concurrent RAEB supports a paraneoplastic nature.
大疱性类天疱疮(BP)与实体器官肿瘤存在公认的关联,但在血液系统恶性肿瘤中相对少见。我们报告一名67岁男性,在被诊断为骨髓增生异常综合征及伴原始细胞增多的难治性贫血(RAEB)后发生了BP。皮肤活检通过直接免疫荧光显示表皮下大疱,揭示沿基底膜有线性C3和IgG沉积。他的BP对传统治疗耐药,仅对高剂量口服泼尼松龙和硫唑嘌呤联合治疗有反应。其病情的相对难治性及并发的RAEB支持副肿瘤性本质。