A 40-year-old woman comes to the physician because of concerns about her legs. She states that she develops a warm burning sensation and redness involving her feet and legs during the night and sometimes when lying on the couch. Her symptoms are worse in the winter when her feet are covered. Elevating her legs and keeping them uncovered either prevents or reduces her symptoms. She considers herself to be in good health otherwise and has no other symptoms.
Physical examination including complete neurologic examination is unremarkable.
Laboratory results are as follows:

Which of the following is most likely to relieve this patient's symptoms?

Question

A 40-year-old woman comes to the physician because of concerns about her legs.  She states that she develops a warm burning sensation and redness involving her feet and legs during the night and sometimes when lying on the couch.  Her symptoms are worse in the winter when her feet are covered.  Elevating her legs and keeping them uncovered either prevents or reduces her symptoms.  She considers herself to be in good health otherwise and has no other symptoms.
Physical examination including complete neurologic examination is unremarkable.
Laboratory results are as follows:

Which of the following is most likely to relieve this patient's symptoms?

Quizplus · Accepted Answer

11ec193a_9824_f898_853d_293e98b94abc_MD0002_00 This patient's presentation is consistent with erythromelalgia, a condition that causes burning pain and erythema (sometimes pallor and/or cyanosis) in the extremities. It is likely due to intermittent blood vessel obstruction. Episodes are usually triggered by heat, exertion, stress, insomnia, or pressure. Symptoms can occur in the symmetric extremities (lower > upper), ears, or face. Patients typically avoid wearing socks or shoes since the generated heat can precipitate attacks. Elevating the legs can also help relieve symptoms. Erythromelalgia can be either primary or secondary, and is usually due to an underlying myeloproliferative disorder with platelet counts > 400,000 cells/µL (as seen in this patient). Diagnosis is usually clinical as there are no specific diagnostic tests. Treatment is directed at the underlying disorder (if secondary), but aspirin may reduce symptoms in both primary and secondary erythromelalgia. Aspirin is also helpful in reducing vascular events in patients with essential thrombocythemia. In addition, patients are instructed to elevate their extremities, cool the affected area, and avoid heat or any other triggers. (Choice A) Allopurinol does not treat erythromelalgia; it is given to patients with suspected polycythemia vera and either symptomatic hyperuricemia or high urinary uric acid excretion to prevent uric acid stones. (Choice C) Pregabalin is effective for diabetic neuropathy. Although this patient has an elevated A1C and new type 2 diabetes, diabetic neuropathy usually presents with altered proprioception; loss of vibratory sensation; and impairment of pain, light touch, and temperature. Ankle reflexes are decreased or absent. This patient's normal physical examination makes this unlikely. (Choice D) Prednisone may be indicated for treatment of an underlying disorder (e.g., SLE) in secondary erythromelalgia. However, this patient more likely has essential thrombocytosis which responds to hydroxyurea rather than prednisone. (Choice E) Vitamin B₁₂ deficiency associated neuropathy starts with paresthesias and ataxia. Examination shows loss of vibration and position sense that can progress to severe weakness, spasticity, and paraplegia. Leukopenia, thrombocytopenia (rather than thrombocytosis), and anemia are associated with cobalamin deficiency. Educational objective: Erythromelalgia usually presents with recurrent episodic warmth, redness, and burning in the extremities symmetrically. Secondary erythromelalgia usually indicates an underlying myeloproliferative disorder. Treatment is directed towards the underlying disorder in secondary erythromelalgia, and aspirin may relieve symptoms in most patients. __________ References: Natural history of erythromelalgia: presentation and outcomes in 168 patients. (http://www.ncbi.nlm.nih.gov/pubmed/10724194) Erythromelalgia and vascular complications in polycythemia vera. (http://www.ncbi.nlm.nih.gov/pubmed/9387203) Erythromelalgia (http://www.ncbi.nlm.nih.gov/pubmed/15075045)

A 40-Year-Old Woman Comes to the Physician Because of Concerns