erythromelalgia
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GC: n

S: NHS – https://www.nhs.uk/conditions/erythromelalgia/ (last access: 8 December 2024); CC – https://my.clevelandclinic.org/health/diseases/22752-erythromelalgia (last access: 8 December 2024).

N: 1. The etymology of its name comes from the Greek: erythros “red”, melos “limb” and algos “pain”.

2. Also known as: erythermalgia, erythralgia.

  • erythromelalgia, rare disease in which the blood vessels of the hands and feet go through spasms of dilation associated with burning pain, increased skin temperature, and redness. The disease may be primary (in which case the cause is unknown), or secondary (caused by underlying disorders of the nervous system, blood, or vascular system). It may be exacerbated by external heat and exercise. Treatment includes rest, elevation of the extremity, and cold applications. Aspirin can be used to relieve pain.

3. 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.

4. 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.

S: 1. NIH – https://pmc.ncbi.nlm.nih.gov/articles/PMC5871369/ (last access: 8 December 2024). 2. EncBrit – https://www.britannica.com/science/erythromelalgia (last access: 8 December 2024). 3 & 4. NIH – https://pmc.ncbi.nlm.nih.gov/articles/PMC5871369/ (last access: 8 December 2024).

SYN: erythermalgia, erythralgia. (depending on context)

S: EncBrit – https://www.britannica.com/science/erythromelalgia (last access: 8 December 2024)

CR: thrombocytopenia