Klejtman T, Garel B, Senet P, Tribout L, Bachmeyer C, Barbaud A, Monfort J-B
Service de dermatologie et allergologie, université Pierre-et-Marie-Curie, Paris VI, hôpital Tenon, AP-HP, 4, rue de la Chine, 75020 Paris, France.
Service de dermatologie et allergologie, université Pierre-et-Marie-Curie, Paris VI, hôpital Tenon, AP-HP, 4, rue de la Chine, 75020 Paris, France.
Ann Dermatol Venereol. 2018 Dec;145(12):761-764. doi: 10.1016/j.annder.2018.07.010. Epub 2018 Sep 7.
Digital necrosis is rarer than lower limb necrosis and constitutes a medical or surgical emergency. Etiological evaluation is required. Cold agglutinin disease is a cause of digital necrosis but diagnosis is difficult.
Herein we report the case of a 57-year-old man presenting recent paroxysmal acrosyndrome of the left hand subsequently complicated by digital necrosis following occupational exposure to cold in his work as a forklift driver. After etiological evaluation, a diagnosis of primary cold agglutinin disease was made. Intravenous rituximab and topical treatment resulted in complete healing.
Cold agglutinin disease is a rare type of auto-immune hemolytic anemia. Following exposure to cold, paroxysmal cutaneous signs are frequent. The disease may be either primary or secondary with B-cell lymphoproliferative disorder, auto-immune disease or infection. A thorough workup is required. To date, the treatment combining the best positive response rate and good safety is rituximab in weekly perfusions over a 1-month period.
手指坏死比下肢坏死少见，属于内科或外科急症，需要进行病因评估。冷凝集素病是手指坏死的一个病因，但诊断困难。
在此我们报告一例57岁男性病例，该患者近期出现左手阵发性肢端综合征，随后在其叉车司机工作中因职业性接触寒冷而并发手指坏死。经过病因评估，确诊为原发性冷凝集素病。静脉注射利妥昔单抗及局部治疗后实现完全愈合。
冷凝集素病是一种罕见的自身免疫性溶血性贫血。接触寒冷后，阵发性皮肤症状较为常见。该病可能是原发性的，也可能继发于B细胞淋巴增殖性疾病、自身免疫性疾病或感染。需要进行全面检查。迄今为止，具有最佳阳性反应率且安全性良好的治疗方法是在1个月内每周输注利妥昔单抗。
