Histologic Findings

In patients with thrombocythemia, skin biopsy results have shown arteriolar endothelial cell swelling, with sparing of venules, capillaries, and nerves. Thickening of the vessel wall and narrowing of the lumen occur as a consequence of smooth muscle cell proliferation. Thickened arterioles may contain occlusive thrombi and ultimately may become fibrotic.

Biopsies from patients with primary erythromelalgia show mild mononuclear perivascular infiltrates with edema, thickened vascular basement membranes, and moderate endothelial swelling. The intimal thickening and thrombi seen in secondary erythromelalgia are lacking.

Laboratory Studies

A complete blood count (CBC) with differential should be obtained to search for evidence of a myeloproliferative disorder. In particular, it is important to look for a platelet count higher than 600 × 10⁹/L (600 × 10³ µg/L), a hematocrit greater than 50%, or an elevated granulocyte count (with all stages of maturation). Other laboratory results are nonspecific.

Imaging Studies

No specific findings are present on plain radiography of the feet and hands. Triple-phase technetium bone scanning is warranted for patients with a history of trauma or stroke and those in whom complex regional pain syndrome type 1 (reflex sympathetic dystrophy) is a strong possibility (particularly if the symptoms are unilateral).

Other Tests

Thermography reveals elevated skin temperatures in the affected areas, but this finding is not necessary to establish the diagnosis. Striking differences in surface temperature can occur between involved and uninvolved areas.

Treatment & Management

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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