Erythromelalgia: a cutaneous manifestation of neuropathy?

Abstract

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.

Figure 1

Physiology of skin pain. Cutaneous nociceptors play a fundamental role in the reception and transmission of painful stimulus. They are stimulated by different factors and in turn involved in neurogenic inflammation.

Figure 2

Erythema and edema on both hands, more pronounced on the right

Figure 3

Combined erythema and edema due to foot erythromelalgia

Figure 4

Acrocyanosis on both hands with slight livedo reticularis in the dorsal aspect. Feet are not compromised

Figure 5

Red nails on hand