Acute adult-onset still's disease presenting as pulmonary hemorrhage, urticaria, angioedema and leukemoid reaction: a case report and literature review.
Acute adult-onset still's disease presenting as pulmonary hemorrhage, urticaria, angioedema and leukemoid reaction: a case report and literature review.
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DOI：
10.1186/s40064-015-0924-8
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发表时间：
2015
期刊：
影响因子：
--
通讯作者：
Quintana G
中科院分区：
文献类型：
--
作者：
Mora Alfonso SA;Rodríguez DM;Londoño JD;Valle-Oñate R;Quintana G
成人型斯蒂尔病是一种病因不明的罕见全身性炎症性疾病，其特征是持续高热、关节疼痛和独特的鲑鱼色凹凸不平的皮疹的经典三联征，然而，可存在多器官受累。一位40岁的健康女性因7天高烧和全身关节痛被转诊到我院，体格检查显示在足底、手掌和舌头上检测到血管神经性水肿，以及广泛的对称分布的红色荨麻疹斑块，影响手臂、手背、上下胸部和背部。5天后发热，患者出现呼吸困难、咳嗽和低氧血症，影像学检查显示单侧实变和胸腔积液。支气管肺泡灌洗和皮肤活检结果与嗜酸性荨麻疹一致。排除血液系统疾病、感染及其他自身免疫性疾病。成人斯蒂尔病的诊断可能非常困难。有没有具体的测试和依赖通常是放在一个复杂的症状和良好的描述，在大多数患者中看到的典型皮疹。近年来，然而，其他皮肤表现的成人发病斯蒂尔病已被报道，但这些都不是那么众所周知的。罕见表现的证据越来越多，成人型斯蒂尔病的早期临床表现非常多变，使诊断困难。出于这个原因，关于该疾病的早期临床表现的数据是有意义的。我们报告了第一例急性成人斯蒂尔病与肺出血，荨麻疹和血管性水肿，包括一种罕见的全身表现为白血病反应。
Adult-onset Still’s disease is a rare systemic inflammatory disorder of unknown aetiology characterized by the classic triad of persistent high spiking fevers, joint pain and a distinctive salmon-colored bumpy rash however, the multiorgan involvement can be present. A 40-year-old woman previously healthy was referred to our hospital with 7 days of high fever and generalized arthralgia, The physical exam revealed angioneurotic edema detected on soles, palms and tongue and widespread red, urticated plaques in a symmetrical distribution affecting the arms, dorsal hands, upper and lower chest and back. Followed 5 days later by fever, the patient presented dyspnea, cough and hypoxemia, the imaging studies showed unilateral consolidation and pleural effusion. The bronchoscopy with bronchoalveolar lavage and skin biopsy were consistent with neutrophilic urticarial. The hematological disorders, infections and other autoimmune diseases were excluded. The diagnosis of adult-onset Still’s disease can be very difficult. There are no specific tests and reliance is usually placed on a symptom complex and the well described typical rash seen in most patients. In recent years, however, other cutaneous manifestations of Adult-onset Still’s disease have been reported but these are not so well known. The evidence of rare manifestations is growing and the early clinical presentation of Adult-onset Still’s is extremely variable, making diagnosis difficult. For this reason, data on early clinical presentation of the disease are of interest. We reported the first case of acute Adult-onset Still’s disease with the association of pulmonary hemorrhage, urticaria and angioedema including a rare systemic manifestation as leukemoid reaction.
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影响因子：
1.6
作者：
POUCHOT, J;SAMPALIS, JS;ESDAILE, JM
通讯作者：
ESDAILE, JM
影响因子：
3.4
作者：
Salaffi, F;Filosa, G;Maestrini, MD
通讯作者：
Maestrini, MD
影响因子：
--
作者：
Hawkins, PN;Lachmann, HJ;McDermott, MF
通讯作者：
McDermott, MF
DOI：
10.1111/j.1468-3083.2004.01142.x
发表时间：
2005-05-01
影响因子：
9.2
作者：
Affleck, AG;Littlewood, SM
通讯作者：
Littlewood, SM
影响因子：
4.5
作者：
Sari I;Birlik M;Binicier O;Akar S;Yilmaz E;Onen F;Akkoc N
通讯作者：
Akkoc N