eMedicine Specialties > Hematology > Stem Cells and Disorders

Erythromelalgia: Differential Diagnoses & Workup

Author: 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
Coauthor(s): Andrea LM Silber, MD, Associate Clinical Professor of Medicine, Section of Hematology-Oncology, Yale University School of Medicine
Contributor Information and Disclosures

Updated: Aug 13, 2009

Differential Diagnoses

Cellulitis
Frostbite
Reflex Sympathetic Dystrophy

Other Problems to Be Considered

Fabry disease
Peripheral neuropathy
Raynaud phenomenon
Vasculitis

Workup

Laboratory Studies

  • On the CBC with differential, look for evidence of a myeloproliferative disorder. In particular, look for a platelet count greater than 600 X 109/L (600 X 103 mcg/L) but also a hematocrit greater than 0.50 (>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 with patients with a history of trauma or stroke and when reflex sympathetic dystrophy is strongly considered (particularly if the symptoms are unilateral).

Other Tests

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

Histologic Findings

In patients with thrombocythemia, skin biopsy results have shown arteriolar endothelial cell swelling, with sparing of venules, capillaries, and nerves. A thickening of the vessel wall and luminal narrowing due to smooth muscle cell proliferation occur. Thickened arterioles may contain occlusive thrombi and ultimately may become fibrosed. Biopsy specimens in 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.

More on Erythromelalgia

Overview: Erythromelalgia
Differential Diagnoses & Workup: Erythromelalgia
Treatment & Medication: Erythromelalgia
Follow-up: Erythromelalgia
Multimedia: Erythromelalgia
References
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References

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Further Reading

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Keywords

erythromelalgia, erythermalgia, primary erythromelalgia, secondary erythromelalgia, myeloproliferative disorder, arteriolar fibrosis, idiopathic erythromelalgia, platelet-mediated erythromelalgia, aspirin-sensitive erythromelalgia, primary erythermalgia, secondary erythermalgia, polycythemia vera, essential thrombocytosis, peripheral vascular disease

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Contributor Information and Disclosures

Author

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.

Coauthor(s)

Andrea LM Silber, MD, Associate Clinical Professor of Medicine, Section of Hematology-Oncology, Yale University School of Medicine
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: sanofi Aventis Honoraria Speaking and teaching; novartis Honoraria Speaking and teaching; gsk Honoraria Speaking and teaching

Medical Editor

Rodger L Bick, MD, PhD, FACP, Clinical Professor of Medicine, University of Texas Southwestern Medical Center; Director, Dallas and Pacific Thrombosis Hemostasis and Vascular Medicine Clinical Center
Rodger L Bick, MD, PhD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Blood Banks, American Cancer Society, American College of Angiology, American College of Physicians, American Geriatrics Society, American Heart Association, American Medical Association, American Society for Clinical Pathology, American Society of Hematology, Association of Clinical Scientists, California Medical Association, California Thoracic Society, International College of Angiology, International Society of Hematology, International Society on Thrombosis and Haemostasis, New York Academy of Sciences, and Southwest Oncology Group
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Troy H Guthrie, Jr, MD, Director of Cancer Institute, Baptist Medical Center
Troy H Guthrie, Jr, MD is a member of the following medical societies: American Federation for Medical Research, American Medical Association, American Society of Hematology, Florida Medical Association, Medical Association of Georgia, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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