Morshedi Bijan, Ring Kari
University of Virginia Health System, 1215 Lee St, Charlottesville, VA 22903, United States.
Gynecol Oncol Rep. 2020 Jan 18;32:100534. doi: 10.1016/j.gore.2019.100534. eCollection 2020 May.
Pemphigus is a group of autoimmune intraepidermal blistering diseases caused by immunoglobulins directed against keratinocyte cell surface components. In this case report, we identify a non-classical paraneoplastic pemphigus (PNP) foliaceous related to an undifferentiated uterine sarcoma. The patient is a 54-year-old Chinese female with a past medical history of arthritis who presented with worsening fatigue in November 2017 and an itchy, blistering, erythematous annular plaque that first appeared on her chest in February 2018. Given high suspicion for primary immunobullous disease despite negative immunofluorescence and lack of subepidermal split on initial biopsy, a repeat biopsy was performed from the right thigh showing positive intraepidermal "net-like" staining for C3 and IgG, but was negative for IgA, IgM, and fibrinogen. IgG antibodies against desmoglein 1 were elevated at 280u (reference range <18), but none resulted against desmoglein 3, consistent with pemphigus foliaceus. This patient's PNP was resistant to treatment with azathioprine, dapsone, mupirocin cream, or betamethasone ointment, but responded to prednisone and rituximab per lymphoma protocol at 375 mg/m weekly for one month in December 2018. In February 2019, the patient had 2-3 episodes of postmenopausal vaginal bleeding and subsequent hysteroscopy with dilation and curettage revealed an undifferentiated uterine sarcoma. The patient underwent an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy, and bilateral pelvic lymph node sampling. After surgical staging, she noted significant improvement in her baseline skin lesions and has had no new lesions since surgery. Repeat desmoglein antibodies showed anti-Dsg1 antibodies of 32u (reference range <18) and anti-Dsg3 antibodies of 1u (reference range <19), as compared to the anti-Dsg1 antibodies of 280u in June 2018. She has since completed 4 cycles of adjuvant gemcitabine and docetaxel for her stage IIB undifferentiated uterine sarcoma with no recurrence of the pemphigus lesions.
天疱疮是一组自身免疫性表皮内水疱性疾病，由针对角质形成细胞表面成分的免疫球蛋白引起。在本病例报告中，我们确定了一例与未分化子宫肉瘤相关的非典型副肿瘤性落叶型天疱疮（PNP）。患者为一名54岁的中国女性，有骨关节炎病史，2017年11月出现疲劳加重，2018年2月胸部首次出现瘙痒、水疱、红斑性环状斑块。尽管初始活检免疫荧光阴性且无表皮下裂隙，但高度怀疑原发性免疫性大疱病，遂从右大腿再次活检，显示表皮内C3和IgG呈阳性“网状”染色，但IgA、IgM和纤维蛋白原呈阴性。抗桥粒芯糖蛋白1的IgG抗体升高至280u（参考范围<18），但抗桥粒芯糖蛋白3抗体均为阴性，符合落叶型天疱疮。该患者的PNP对硫唑嘌呤、氨苯砜、莫匹罗星乳膏或倍他米松软膏治疗耐药，但在2018年12月按照淋巴瘤方案接受泼尼松和利妥昔单抗治疗，剂量为375mg/m²每周一次，共一个月后有反应。2019年2月，患者出现2 - 3次绝经后阴道出血，随后宫腔镜检查及刮宫显示为未分化子宫肉瘤。患者接受了剖腹探查术、全腹子宫切除术、双侧输卵管卵巢切除术及双侧盆腔淋巴结取样。手术分期后，她注意到基线皮肤病变有显著改善，术后未出现新病变。重复检测桥粒芯糖蛋白抗体显示，与2018年6月抗Dsg1抗体280u相比，抗Dsg1抗体为32u（参考范围<18），抗Dsg3抗体为1u（参考范围<19）。此后，她已完成针对IIB期未分化子宫肉瘤的4个周期吉西他滨和多西他赛辅助治疗，天疱疮病变未复发。
