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History

The classic description of erythromelalgia is a triad of redness, pain, and warmth in the extremities, brought on by warming or dependency and relieved by cooling (see the image below).

Cardinal symptoms of erythromelalgia.

Paroxysmal burning pain has also been reported as a presenting symptom of erythromelalgia.^[31]Erythromelalgia has rarely been reported to involve parts other than the extremities (eg, ears, face, genitals).^{[32, 33, 34]}

Symptomatic episodes may last minutes to days. They often begin with an itching sensation and later progress to more severe pain with a burning quality. Pain may be so intense that the patient cannot walk; some must even keep their feet immersed in ice water.

The lower extremities are affected more often than the upper extremities. The soles of feet and toes are most commonly involved. In rare cases, involvement may reach as high as the knees. Involvement is usually bilateral, though not necessarily symmetric. Warming the extremity or placing and maintaining the extremity in a dependent position can exacerbate symptoms; cooling and elevating the extremity can relieve symptoms.

Raynaud phenomenon has been reported to occur between episodes of erythromelalgia, but this may be coincidental.

In cases associated with a myeloproliferative disorder, erythromelalgia usually precedes the diagnosis of the myeloproliferative disorder by a median of 2.5 years. Rapid relief with aspirin is typical of this type of erythromelalgia and can be supportive diagnostically.

Given the association with myeloproliferative disorders, the clinician should inquire about other possible clinical manifestations of myeloproliferative disorders, such as:

Pruritus
Thrombotic episodes (eg, transient visual loss)
Constitutional symptoms
Abdominal discomfort

Physical Examination

Between episodes, examination findings may be normal.

The affected extremity becomes warm and tender during an episode and appears dusky, red, and sometimes mottled.

Peripheral pulses may be normal or bounding. Acrocyanosis may be observed; rarely, it progresses to distal digital necrosis. Ischemic ulcers may be observed and may become infected secondarily.

Checking for splenomegaly and lymphadenopathy should be part of a comprehensive physical examination in a patient with suspected erythromelalgia.

Complications

Complications appear to be more frequent in patients who clearly have platelet-mediated disease.

Ulceration, necrosis, and gangrene of affected extremities are possible. Digital necrosis or skin ueration with secondary infection can lead to amputation. At least one patient had near-fatal hypothermia related to constant cooling required to control symptoms.

Differential Diagnoses

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Media Gallery

Cardinal symptoms of erythromelalgia.

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Author

Drew H Barnes, MD Fellow Physician, Hospice and Palliative Medicine, The Ohio State University Wexner Medical Center

Drew H Barnes, MD is a member of the following medical societies: American Academy of Hospice and Palliative Medicine, American College of Physicians, American Medical Association, American Society of Clinical Oncology, American Society of Hematology

Disclosure: Nothing to disclose.

Coauthor(s)

Claudia M da Costa Dourado, MD Clinical Assistant Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Program Director, Hematology and Medical Oncology Fellowship Program, Attending Physician, Division of Hematology and Medical Oncology, Department of Medicine, Einstein Medical Center Philadelphia

Claudia M da Costa Dourado, MD is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Ali T Taher, MD, PhD, FRCP Professor of Medicine, Associate Chair of Research, Department of Internal Medicine, Division of Hematology/Oncology, Director of Research, NK Basile Cancer Center, American University of Beirut Medical Center, Lebanon

Disclosure: Nothing to disclose.

Antoine N Saliba, MD Post-Doctoral Research Fellow, Division of Hematology/Oncology, Department of Internal Medicine, American University of Beirut Medical Center

Disclosure: Nothing to disclose.

Acknowledgements

Robert J Nardino, MD Director, Internal Medicine Residency Program, Assistant Clinical Professor, Department of Internal Medicine, Hospital of Saint Raphael, Yale University School of Medicine

Robert J Nardino, MD is a member of the following medical societies: American College of Physicians, Association of Program Directors in Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Andrea LM Silber, MD Medical Oncologist, Father Michael J McGivney Center for Cancer Care, Saint Raphael Hospital

Andrea LM Silber, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American Society of Preventive Oncology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

Erythromelalgia Clinical Presentation

History

Physical Examination

Complications

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