Knowra Erythromelalgia Erythromelalgia Erythromelalgia is a rare disorder marked by episodes of burning pain, redness, and increased skin temperature, usually affecting the feet or hands. Symptoms often worsen with heat or exercise and ease with cooling.
Small fiber neuropathy : Damage or dysfunction of thinly myelinated and unmyelinated nerve fibers that carry pain, temperature, and autonomic signals. Small-fiber dysfunction is implicated in the burning pain and abnormal temperature regulation of erythromelalgia.
Clinical diagnosis : Identification of a disease through symptoms, examination, and medical history, sometimes supported by tests. Erythromelalgia is diagnosed chiefly from its characteristic episodic symptoms and examination.
Primary erythromelalgia : Erythromelalgia occurring without an identified associated blood disorder or other underlying condition. This category includes inherited cases and cases without a known cause.
Raynaud phenomenon : Episodes of reduced blood flow to the fingers or toes, often causing pallor, bluish discoloration, and pain after cold exposure. Raynaud attacks are typically triggered by cold and involve vasoconstriction, unlike the heat-triggered redness of erythromelalgia.
Cutaneous vasodilation : Widening of blood vessels in the skin, increasing local blood flow and often skin temperature. Excess skin blood flow during attacks contributes to redness and warmth.
Differential diagnosis : The process of distinguishing a condition from other disorders with similar features. Cellulitis, gout, and vascular disorders can resemble an erythromelalgia attack.
Secondary erythromelalgia : Erythromelalgia that occurs in association with another disease, medication, or medical condition. Identifying an associated cause can change treatment and symptom control.
Complex regional pain syndrome : A chronic pain condition, usually affecting a limb, with sensory, autonomic, and sometimes motor changes. It can cause limb pain, warmth, and redness, but typically follows injury and has a broader persistent pattern.
SCN9A : A gene encoding Nav1.7, a voltage-gated sodium channel expressed in pain-sensing neurons. Gain-of-function variants in SCN9A cause some inherited cases by making pain neurons hyperexcitable.
Cooling therapy : Use of measures that lower tissue temperature to relieve heat-related symptoms. Cooling often eases attacks, but prolonged ice-water exposure can injure skin.
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