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.