Erythromelalgia may be associated with a number of underlying conditions. Identifying the cause can be helpful for planning management and avoiding triggering factors.


Conditions associated with erythromelalgia:

(1) familial (inherited as an autosomal dominant condition)

(2) secondary to myeloproliferative disease, usually essential thrombocythemia or polycythemia rubra vera

(3) secondary to drugs (nifedipine, bromocriptine)

(4) secondary to pregnancy

(5) secondary to connective tissue diseases or vasculitis

(6) secondary to gout (gout may be associated with hypercoagulability)

(7) secondary to diabetes mellitus (questionable, since this could be due to neurological or vascular complications)

(8) secondary to neurological disorders including multiple sclerosis (need to exclude reflex dystrophy)

(9) paraneoplastic

(10) associated with HIV (according to Mork and Kvernebo, 2000)

(11) thrombotic thrombocytopenic purpura (TTP)

(12) idiopathic


In patients where the condition is associated with thrombocythemia and microthrombosis (thrombocythemia, other myeloproliferative disorders):

(1) the administration of low dose aspirin may be associated with a prompt relief of symptoms lasting 3-4 days.

(2) peripheral gangrene may occur


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