Erythromelalgia is a rare condition causing the distal extremities to be red, hot and painful. It may be associated with a variety of conditions and has an increased morbidity and mortality. The quality of life may be affected by the steps taken to avoid an attack or to achieve relief.


Clinical triad:

(1) redness/erythema of the skin in the affected areas ("erythros"), which may be mottled

(2) increased temperature of the affected skin

(3) pain ("algos"), usually in the extremities ("melos"), often described as burning. The onset of pain may be preceded by a feeling of prickling or itching or "pins and needles". Some patients notice a feeling of numbness.


Thompson et al (1979) included 2 more features (pentad):

(4) pain aggravated by warming or exercise (in 51% and 29% respectively according to Davis) or during febrile illness. Wearing shoes may precipitate an attack in some patients.

(5) pain relieved by cooling (in 67% according to Davis), rest and/or elevation


Distribution: may be symmetrical or asymmetrical, with some patients having unilateral disease:

(1) distal foot or sole of foot and one or more toes

(2) one or more fingers and toes

(3) one or more fingers

(4) lower legs and feet

(5) feet and hands


Symptoms may be relieved by exposure to cold.


Differential diagnosis:

(1) cellulitis

(2) polyneuropathy

(3) reflex dystrophy

(4) arterial or venous insufficiency

(5) Raynaud's phenomenon


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