Leroux María Bibiana
Private practice - Rosario, Argentina.
An Bras Dermatol. 2018 Jan-Feb;93(1):86-94. doi: 10.1590/abd1806-4841.20187535.
The low prevalence of erythromelalgia, classified as an orphan disease, poses diagnostic and therapeutic difficulties. The aim of this review is to be an update of the specialized bibliography. Erythromelalgia is an infrequent episodic acrosyndrome affecting mainly both lower limbs symmetrically with the classic triad of erythema, warmth and burning pain. Primary erythromelalgia is an autosomal dominant inherited disorder, while secondary is associated with myeloproliferative diseases, among others. In its etiopathogenesis, there are neural and vascular abnormalities that can be combined. The diagnosis is based on exhaustive clinical history and physical examination. Complications are due to changes in the skin barrier function, ischemia and compromise of cutaneous nerves. Because of the complexity of its pathogenesis, erythromelalgia should always be included in the differential diagnosis of conditions that cause chronic pain and/or peripheral edema. The prevention of crisis is based on a strict control of triggers and promotion of preventive measures. Since there is no specific and effective treatment, control should focus on the underlying disease. However, there are numerous topical and systemic therapies that patients can benefit from.
红斑性肢痛症患病率较低，被归类为罕见病，这给诊断和治疗带来了困难。本综述的目的是更新专业文献。红斑性肢痛症是一种罕见的发作性肢端综合征，主要对称累及双下肢，具有红斑、发热和灼痛这一典型三联征。原发性红斑性肢痛症是一种常染色体显性遗传性疾病，而继发性红斑性肢痛症则与骨髓增殖性疾病等有关。在其发病机制中，存在可相互关联的神经和血管异常。诊断基于详尽的临床病史和体格检查。并发症是由皮肤屏障功能改变、缺血和皮神经受损所致。由于其发病机制复杂，红斑性肢痛症应始终纳入导致慢性疼痛和/或外周水肿病症的鉴别诊断中。发作的预防基于对诱发因素的严格控制和预防措施的推广。由于没有特效治疗方法，治疗应侧重于基础疾病。然而，有许多局部和全身治疗方法可供患者受益。
