Balaja Warren, Schmidt Paul, Fenando Ardy
Department of Rheumatology & Internal Medicine, University of Kansas School of Medicine, Kansas City, KS, USA.
SAGE Open Med Case Rep. 2023 Aug 22;11:2050313X231191899. doi: 10.1177/2050313X231191899. eCollection 2023.
A female in her 60s presented to the allergy and immunology clinic for further investigation of ongoing dermatitis. She presented with chronic acrocyanosis, mainly in her left lower extremity, extending distally from her mid thigh with concurrent ulcerations in her foot resulting in immobility secondary to pain. She experienced these symptoms for years without a definitive diagnosis. The lack of diagnosis was due, in part, to her atypical symptoms and laboratory findings that required a high level of clinical suspicion to diagnose. Extensive autoimmune workup was largely unrevealing with the exception of a cold agglutinin titer of 1:250 and a positive anticomplement C3b direct antiglobulin test. A diagnosis of cold agglutinin disease was made and treatment with rituximab monotherapy was initiated.
一名60多岁的女性因持续性皮炎前往过敏与免疫诊所进行进一步检查。她表现为慢性手足发绀，主要累及左下肢，从大腿中部向远端延伸，足部同时伴有溃疡，因疼痛而行动不便。她出现这些症状多年，一直未得到明确诊断。诊断困难部分是由于她的症状和实验室检查结果不典型，需要高度的临床怀疑才能确诊。除了冷凝集素效价为1:250和抗补体C3b直接抗球蛋白试验呈阳性外，广泛的自身免疫检查大多无异常发现。最终诊断为冷凝集素病，并开始使用利妥昔单抗单药治疗。
