Rare process of extremity redness, burning pain, and warmth.

Characterized by intermittent hot and red extremities, with more involvement in the lower extremities than the upper extremities.

Involvement is usually symmetrical.

Associated pain may be severe and debilitating.

Primary and secondary forms have been described.

Polycythemia rubra vera and other myeloproliferative disorders may underlie the secondary form.

Maybe a form of small fiber neuropathy ( Davis MD).

Diabetes is not associated with this entity.

Primary and secondary types exist.

Primary type is idiopathic.

Secondary type related primarily to myeloproliferative disease.

Usually precedes the diagnosis of a myeloproliferative disorder by a median of 2.5 years.

Myeloproliferative disorder of polycythemia and essential thrombocytosis most commonly involved.

This process manifests before the diagnosis of the myeloproliferative disease 85% of the time.

Improvement of symptoms with the use of aspirin is typical of this process induced by a myeloproliferative disorder.

Secondary type may be due to neurologic, hematologic or vascular abnormalities.

In cases with an associated thrombocytosis skin biopsy reveals arteriolar fibrosis and occlusion with platelet thrombi.

Platelet associated disease may be related to enhanced aggregation of platelets, decreased platelet survival with consumption and prostaglandin and cyclooxygenase playing a role.

Factors contributing to primary disease include postganglionic sympathetic dysfunction, C fiber hypersensitivity and arteriovenous shunting with maldistribution of skin perfusion.

Linked to chromosome 2q in some families.

Prevalence in patients with myeloproliferative disorders is 3-65%

May lead to digital necrosis.

Associated with decreased survival.

Male:female in primary disease is 1:2.5.

Secondary form male:female 3:2.

Median age of early onset disease 10 years.

Adult onset most cases occur in the fifth and sixth decades.

Symptoms relieved by cooling or elevating the legs.

Episodes last minutes to days.

Symptoms often begin with itching and progresses to burning pain.

May be bought on by dependency.

Peripheral pulses may be normal or even full and bounding.

May lead to ischemic ulcerations.

Treatment may include: aspirin, nonsteroidal anti-inflammatory drugs, antidepressants and gabapentin.

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