Soria A, Matichard E, Descamps V, Crickx B
Service de Dermatologie, Université Paris 7-Denis-Diderot, Hôpital Bichat, AP-HP, Paris.
Ann Dermatol Venereol. 2007 Apr;134(4 Pt 1):353-6. doi: 10.1016/s0151-9638(07)89190-3.
Acquired hemophilia is a rare disease. Associated pemphigoid is extremely uncommon. We report a case of acquired hemophilia in the course of this dermatosis illustrating the severity of the disease.
An 83 year-old woman was followed for 3 years for pemphigoid treated with topical corticosteroids following a recent relapse. She was hospitalized for surgical treatment of extensive and painful hematoma of the anterior aspect of the right leg following a fall. Preoperative clotting tests showed an extremely long cephalin coagulation time (> 2 x normal) not corrected by addition of control plasma, with a normal prothrombin time, due to acquired hemophilia type A and the presence of factor VIII inhibitor (17 Bethesda units/ml). The coagulant activity of factor VIII was reduced by 3%. The patient was treated with recombinant human factor VII (NovoSeven) and systemic corticosteroids were subsequently administered at a dose of 1 mg/kg/d to ensure direct action on antibody production. Seven days later, the patient presented intense abdominal pain and extension deficit in the right lower limb. An abdominal-pelvic scan revealed spontaneous hematoma of the right psoas-iliac muscle. Despite replacement therapy with NovoSeven and oral corticosteroids, worsening of the hematoma occurred, complicated by hemorrhagic shock, resulting in death.
Acquired hemophilia, as revealed by cutaneous-mucosal bleeding, is a rare disease (1 to 4 cases per million subjects) more commonly seen in adults. It is associated with the presence of antibodies directed against factor VIII. Its complications, particularly hemorrhagic, are fatal in 15 to 20% of cases. While acquired hemophilia seems to occur in isolation in one of every two cases, it may be associated with autoimmune diseases, lymphoproliferative syndromes, solid tumors, the post-partum period, or use of certain drugs. In dermatology, acquired hemophilia has been reported in association with pemphigoid (9 cases), in which case the prognosis is consistently very poor, with pemphigus vulgaris (5 cases), more recently with acquired epidermolysis bullosa (3 cases), and finally with mucosal pemphigoid (1 case).
Given the severity of associated hemorrhagic accidents, early identification of this clotting disorder is warranted in order to allow initiation of treatment as soon as possible.
获得性血友病是一种罕见疾病。相关类天疱疮极为罕见。我们报告一例在这种皮肤病病程中出现的获得性血友病病例，以说明该疾病的严重性。
一名83岁女性因类天疱疮接受局部皮质类固醇治疗，近期复发后随访3年。她因跌倒后右小腿前部出现广泛且疼痛的血肿而住院接受手术治疗。术前凝血检查显示白陶土凝血时间极长（>正常对照值的2倍），加入正常对照血浆后未纠正，凝血酶原时间正常，诊断为获得性A型血友病且存在凝血因子VIII抑制物（17贝塞斯达单位/毫升）。凝血因子VIII的凝血活性降低了3%。患者接受重组人凝血因子VII（诺其）治疗，随后给予1毫克/千克/天剂量的全身皮质类固醇以确保对抗体产生有直接作用。7天后，患者出现剧烈腹痛和右下肢伸展功能障碍。腹部盆腔扫描显示右腰大肌-髂肌自发性血肿。尽管使用诺其和口服皮质类固醇进行替代治疗，但血肿仍恶化，并并发失血性休克，导致患者死亡。
获得性血友病以皮肤黏膜出血为表现，是一种罕见疾病（每百万人口中1至4例），在成年人中更为常见。它与针对凝血因子VIII的抗体存在有关。其并发症，尤其是出血性并发症，在15%至20%的病例中是致命的。虽然每两例获得性血友病中似乎有一例是单独发生的，但它可能与自身免疫性疾病、淋巴增殖综合征、实体瘤、产后或使用某些药物有关。在皮肤病学领域，已报告获得性血友病与类天疱疮相关（9例），这种情况下预后一直很差，与寻常型天疱疮相关（5例），最近与获得性大疱性表皮松解症相关（3例），最后与黏膜类天疱疮相关（1例）。
鉴于相关出血性意外的严重性，有必要尽早识别这种凝血障碍，以便尽快开始治疗。
