Qi Fei, Gao Yimeng, Jin Hongzhong
Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, People's Republic of China.
State Key Laboratory of Complex Severe and Rare Diseases, Beijing, People's Republic of China.
Clin Cosmet Investig Dermatol. 2024 Aug 2;17:1747-1756. doi: 10.2147/CCID.S466449. eCollection 2024.
Livedoid vasculopathy is an uncommon cutaneous ulcerative dermatosis that is challenging to diagnose. Diagnostic delay brought both pain and uncurable atrophied scar to patients.
We conducted this study to identify the factors responsible for the initial misdiagnosis of livedoid vasculopathy and to identify possible methods to increase the diagnostic accuracy of livedoid vasculopathy.
We conducted a retrospective medical record review to confirm the diagnosis of livedoid vasculopathy in patients who visited the Department of Peking Union Medical College Hospital for the first time. We used the Diagnosis Error Evaluation and Research taxonomy to evaluate missed cases.
Twenty-three patients (85.18%) had an alternate diagnosis, including 10 (43.4%) with two or more diagnoses. The average time from disease onset to the final diagnosis of livedoid vasculopathy was 4.61 ± 0.69 years. The major diagnostic errors were clinician assessment failures and failures in the timely follow-up and rechecking of patients. Allergic vasculitis was the most common misdiagnosis. Other alternate diagnoses include Henoch-Schoenlein purpura, pigmented purpuric dermatosis, eczema, erythema nodosum, and reactive perforating collagenases. Twenty-three patients (65.21%) received systemic corticosteroid therapy before the final diagnosis of livedoid vasculopathy.
It is critical to raise the awareness of clinicians about livedoid vasculopathy, especially when patient present with extensive livedo racemosa or long-lasting purpuric lesions on the lower limbs. Long-term follow-up is necessary, especially for younger patients. Skin biopsy is recommended before systematic therapy.
萎缩性硬化性苔藓是一种罕见的皮肤溃疡性皮肤病，诊断具有挑战性。诊断延迟给患者带来了疼痛和无法治愈的萎缩性瘢痕。
我们开展本研究以确定导致萎缩性硬化性苔藓初始误诊的因素，并确定提高萎缩性硬化性苔藓诊断准确性的可能方法。
我们进行了一项回顾性病历审查，以确认首次就诊于北京协和医院皮肤科的患者中萎缩性硬化性苔藓的诊断。我们使用诊断错误评估和研究分类法来评估漏诊病例。
23例患者（85.18%）有其他诊断，其中10例（43.4%）有两种或更多诊断。从疾病发作到最终诊断为萎缩性硬化性苔藓的平均时间为4.61±0.69年。主要诊断错误是临床医生评估失败以及对患者的随访和复查不及时。过敏性血管炎是最常见的误诊。其他替代诊断包括过敏性紫癜、色素性紫癜性皮肤病、湿疹、结节性红斑和反应性穿通性胶原病。23例患者（65.21%）在最终诊断为萎缩性硬化性苔藓之前接受了全身糖皮质激素治疗。
提高临床医生对萎缩性硬化性苔藓的认识至关重要，尤其是当患者出现广泛的网状青斑或下肢长期紫癜性病变时。长期随访是必要的，尤其是对于年轻患者。在进行系统治疗前建议进行皮肤活检。
