Characterized by hot, red, intensely painful feet or hands – pain is described as burning. More common in feet than hands. Tends to occur in ‘attacks’ that last for minutes to hours to sometimes days, usually following exposure to warm environment. Cooler environments provide relief.
Assumed may be the opposite of Raynauds phenomenon - now thought that these two disorders are more similar than dissimilar. Thought to be triggered by temperature induced release of vasoactive substances
Usually follows a chronic, sometimes progressive and disabling course.
Now considered that it may not be a disease entity but a syndrome of dysfunctional vascular dynamics - recent studies dysfunction is reversible in some patients.
• Secondary – associated with myeloproliferative diseases (eg polycythemia vera); other diseases (eg hypertension, diabetes, rheumatoid arthritis, gout); drugs (eg nifedipine)
Differential diagnosis – regional complex pain syndromes, Buerger’s disease