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eMedicine Journal
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Dermatology
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Diseases Of The Subcutaneous Tissue
Erythema Gyratum Repens |
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 | AUTHOR INFORMATION | Section 1 of 11    |
Authored by Raul DelRosario, MD, Surgical Pathology and Dermatopathology, South Coast Medical
Coauthored by Karen Allen, MD, Consulting Dermatologist; Suzanne Kaneshiro, MD, Staff Physician, Department of Dermatology, University of California at Irvine
Raul DelRosario, MD, is a member of the following medical societies: American Society of Clinical Pathologists
Edited by Carrie L Kovarik, MD, Assistant Professor, Department of Dermatology and Dermatopathology, University of Pennsylvania School of Medicine; Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA; Jeffrey P Callen, MD, Chief, Division of Dermatology, Professor of Medicine (Dermatology), Department of Internal Medicine, University of Louisville School of Medicine; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; and Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
| Author's Email: | Raul DelRosario, MD |  |
|---|---|---|
| Editor's Email: | Carrie L Kovarik, MD |
eMedicine Journal, February 21 2007, VOLUME 8,
Number 2
| INTRODUCTION | Section 2 of 11  |
Background: Erythema gyratum repens (EGR) is a figurate erythema that is believed to be a paraneoplastic process. In addition to other features, characteristic concentric erythematous bands forming a wood-grain appearance help distinguish EGR from other figurate erythemas, such as erythema annulare centrifugum, erythema migrans, and erythema marginatum.
Pathophysiology: The pathogenesis of EGR remains unknown. The following hypotheses have been proposed:
A mechanism explaining the clinically apparent migrating erythema also has been proposed. This model involves a localized ground substance phenomenon. Granulocytes release factors that stimulate proliferating fibroblasts, producing ground substance with increased viscosity. This viscous ground substance serves to impede or "wall off" the tissue spread of inflammatory mediators. In EGR, the advancing erythema may represent the advancement of inflammatory mediators through stroma that is unable to keep them walled off.
Frequency:
Mortality/Morbidity: No specific complications are associated with the skin manifestations of EGR, and the condition alone does not lead to death. Symptoms include intense pruritus, and morbidity and mortality may occur related to the underlying condition.
Race: The disease reportedly occurs predominantly in white persons.
Sex: Male-to-female ratio is 2:1.
Age: EGR usually occurs in patients older than 40 years, with a mean age of 63 years, but it has been reported to occur from age 16-75 years.
| CLINICAL | Section 3 of 11 |
History:
Physical:
Causes: EGR is associated with malignancy in as many as 80% of patients. Among visceral malignancies, the lung is the most common site, followed by the breast, urinary bladder, uterus and/or cervix, GI tract (stomach), and prostate. Most patients with EGR develop the eruption before the symptoms of tumor. The time interval between the eruption of EGR and the detection of the tumor can range from simultaneous presentation to up to 6 years after the rash. EGR also has occurred up to 7 months after the detection of malignancy. EGR is associated with some nonneoplastic conditions, such as pulmonary tuberculosis, lupus erythematosus, CREST (calcinosis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) syndrome, virginal breast hypertrophy, pityriasis rubra pilaris, psoriasis, and as a drug reaction to azathioprine in a patient with type I autoimmune hepatitis. In a few patients, no associated conditions exist.
| DIFFERENTIALS | Section 4 of 11 |
Bullous Pemphigoid
Erythema Annulare Centrifugum
Erythema Multiforme
Glucagonoma Syndrome
Granuloma Annulare
Lupus Erythematosus, Subacute Cutaneous
Lyme Disease
Pityriasis Rubra Pilaris
Psoriasis, Plaque
Tinea Corporis
Urticaria, Chronic
Other Problems to be Considered:
Erythema marginatum
Carriers of chronic granulomatous disease
| WORKUP | Section 5 of 11 |
Lab Studies:
Imaging Studies:
Procedures:
| TREATMENT | Section 6 of 11 |
Medical Care: Topical steroids are of no benefit. Corticosteroid injections may improve the pruritus but reportedly do not change the appearance of the skin eruption.
| FOLLOW-UP | Section 7 of 11 |
Prognosis:
| MISCELLANEOUS | Section 8 of 11 |
Medical/Legal Pitfalls:
| TEST QUESTIONS | Section 9 of 11 |
CME Question 1: Which of the following characterizes erythema gyratum repens (EGR)?
A: Wood-grain appearance
B: Migration of rash up to 1 cm/d
C: Intense pruritus
D: Subsequent clearance of signs and symptoms with resolution of the underlying disease
E: All of the above
The correct answer is E: EGR has distinctive dermatologic manifestations characterized by (1) a wood-grain appearance created by concentric mildly scaling bands of flat-to-raised erythema, (2) a fairly rapid migration of the rash (up to 1 cm/d), (3) intense pruritus, (4) a course of rash that closely mirrors the course of the underlying illness with clearance of the rash and relief of pruritus within 6 weeks subsequent to resolution of the underlying illness, and (5) sites of predilection that include the trunk and extremities. Other associated findings with EGR include ichthyosis and palmoplantar hyperkeratosis.
CME Question 2: Which of the following is the most common visceral malignancy associated with erythema gyratum repens (EGR)?
A: Lung cancer
B: Breast cancer
C: Bladder cancer
D: Cervical cancer
E: Prostate cancer
The correct answer is A: EGR is associated with malignancy in up to 80% of patients. Among visceral malignancies, the lung is the most common site, followed by the breast, urinary bladder, uterus and/or cervix, GI tract (stomach), and prostate. Lung cancer has been associated with EGR in as many as 40% of patients.
Pearl Question 1 (T/F): The period between the onset of erythema gyratum repens (EGR) and the detection of malignancy ranges from simultaneous presentation to up to 6 years prior to detection of the malignancy.
The correct answer is True: The onset of EGR often precedes the detection of malignancy by an average of 9 months (range 1-72 mo), although in a minority of patients, EGR occurred simultaneously with or up to 9 months after detection of the neoplasm.
Pearl Question 2 (T/F): Erythema gyratum repens may be seen in nonneoplastic conditions.
The correct answer is True: Erythema gyratum repens also is associated with pulmonary tuberculosis, lupus erythematosus, CREST (calcinosis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, telangiectasia) syndrome, virginal breast hypertrophy, and pityriasis rubra pilaris.
Pearl Question 3 (T/F): A patient with erythema gyratum repens warrants a basic workup.
The correct answer is True: Since there is up to an 80% association with visceral malignancies, it is prudent to perform a workup on a patient for cancer. Workup includes physical exam, screening tests, such as Pap smears for women and prostate-specific antigen (PSA) for men, hematologic workup, blood chemistry, chest x-ray, and Hemoccult test.
Pearl Question 4 (T/F): Erythema gyratum repens usually clears with subsequent resolution of the underlying illness.
The correct answer is True: Usually, clearance of rash and relief of pruritus occurs with subsequent resolution of the underlying illness.
| PICTURES | Section 10 of 11 |
| Caption: Picture 1. Erythema gyratum repens. Courtesy of Jeffrey P. Callen, MD. | |
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| BIBLIOGRAPHY | Section 11 of 11 |
| NOTE: |
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| Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatment therapies daily. The authors, editors, and publisher of this journal have used their best efforts to provide information that is up-to-date and accurate and is generally accepted within medical standards at the time of publication. However, as medical science is constantly changing and human error is always possible, the authors, editors, and publisher or any other party involved with the publication of this article do not warrant the information in this article is accurate or complete, nor are they responsible for omissions or errors in the article or for the results of using this information. The reader should confirm the information in this article from other sources prior to use. In particular, all drug doses, indications, and contraindications should be confirmed in the package insert. FULL DISCLAIMER |
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