Yalcin Arzu Didem, Genc Gizem Esra, Celik Betul, Gumuslu Saadet
Clin Lab. 2014;60(3):523-4. doi: 10.7754/clin.lab.2013.130642.
Herein, we report a case of a man with pruritic bullous pemphigoid (BP) and very high levels of total IgE (5000 kU/L) who was refractory to standard aggressive immunosuppressive regimens (systemic steroids, daily cyclophosphamide) for BP but responded rapidly to systemic anti-IgE (omalizumab). Our patient is a 28 year-old white male. On admission 70% of his body surface area was involved with large bullae overlying urticarial plaques, involving his upper and lower extremities, chest, and abdomen. The circulating level of sCD200 was 48.45 pg/mL in serum and 243 pg/mL in blister fluid. During the second month of follow-up, the patient's sCD200 level decreased to 26.7 pg/mL. After the second round of omalizumab (300 mg), frequency of exacerbations decreased and after the 13th round it had completely disappeared.
在此，我们报告一例患有瘙痒性大疱性类天疱疮（BP）且总IgE水平极高（5000 kU/L）的男性病例。该患者对BP的标准积极免疫抑制方案（全身用类固醇、每日环磷酰胺）治疗无效，但对全身抗IgE（奥马珠单抗）反应迅速。我们的患者是一名28岁的白人男性。入院时，其70%的体表面积出现大片水疱，覆盖于风团样斑块之上，累及上下肢、胸部和腹部。血清中sCD200的循环水平为48.45 pg/mL，水疱液中为243 pg/mL。在随访的第二个月，患者的sCD200水平降至26.7 pg/mL。第二轮奥马珠单抗（300 mg）治疗后，病情加重的频率降低，在第13轮治疗后完全消失。
