Boddu Prajwal, Mohammed Abdul S, Khandelwal Sonali
Advocate Illinois Masonic Medical Center, Department of Internal Medicine, 856 West Nelson Street, Apt No. 2002, Chicago, IL 60657, USA.
Advocate Illinois Masonic Medical Center, Department of Internal Medicine, 2356 North Elston Avenue, No. 306, Chicago, IL 60614, USA.
Case Rep Rheumatol. 2016;2016:4829459. doi: 10.1155/2016/4829459. Epub 2016 Sep 8.
Hypergammaglobulinemic purpura (HGP) is a syndrome constellating recurrent purpura, hypergammaglobulinemia, positive rheumatoid factor (RF), anti-Ro/La antibodies, and elevated erythrocyte sedimentation rate (ESR). We present a case of a 29-year-old female who was diagnosed with Sjogren's syndrome four years prior to presenting with rash on her lower extremities for a period of 6 months. Skin biopsy at the initial visit was consistent with leukocytoclastic vasculitis and was initiated on treatment for it. Her rash evolved into 2-5 mm scattered purpurae while she was on the treatment and a repeat biopsy showed extravasation of RBCs, a sparse mononuclear infiltrate with deposition of plasma cells, and no evidence of leukocytoclastic vasculitis, thus showing a transition from neutrophilic to mononuclear inflammatory vascular disease which is a rare occurrence. Hypergammaglobulinemic purpura sometimes turns out to be a challenging disease to manage and requires an integrated effort from the primary care doctors, rheumatologist, and dermatologist.
高球蛋白血症性紫癜(HGP)是一种综合征,其特征为反复出现紫癜、高球蛋白血症、类风湿因子(RF)阳性、抗Ro/La抗体以及红细胞沉降率(ESR)升高。我们报告一例29岁女性病例,该患者在出现下肢皮疹6个月前4年被诊断为干燥综合征。初诊时的皮肤活检结果符合白细胞破碎性血管炎,并因此开始接受治疗。在治疗期间,她的皮疹演变为2 - 5毫米散在的紫癜,再次活检显示有红细胞外渗、稀疏的单核细胞浸润以及浆细胞沉积,且无白细胞破碎性血管炎的证据,从而显示出从中性粒细胞性炎症性血管病向单核细胞性炎症性血管病的转变,这种情况较为罕见。高球蛋白血症性紫癜有时是一种难以处理的疾病,需要初级保健医生、风湿病学家和皮肤科医生共同努力。