Erythema Gyratum Repens

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Background

Erythema gyratum repens (EGR) is a figurate erythema that is believed to be a paraneoplastic process.[1, 2, 3, 4] In addition to other features, characteristic concentric erythematous bands forming a wood-grain appearance (see the image below) help distinguish erythema gyratum repens from other figurate erythemas, such as erythema annulare centrifugum, erythema migrans, and erythema marginatum.[5]



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Erythema gyratum repens. The characteristic wood-grain pattern is composed of annular, erythematous concentric bands lined by a trailing edge of scale....

Pathophysiology

The pathogenesis of erythema gyratum repens 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 erythema gyratum repens, the advancing erythema may represent the advancement of inflammatory mediators through stroma that is unable to keep them walled off.

Another hypothesis suggests that glutamine contributes to the unusual skin patterns seen in erythema gyratum repens, given the evidence of elevated glutamine levels in the body as a consequence of tumor activity and glutamine self-assembled ring formations when in aqueous solutions.[6]

Etiology

Erythema gyratum repens is associated with malignancy in as many as 80% of patients. Among visceral malignancies, the lung is the most common site,[7, 8, 9] followed by the breast, urinary bladder, uterus and/or cervix, GI tract (stomach), and prostate.[10] Erythema gyratum repens has also been associated with anal cancer.[11] Most patients with erythema gyratum repens develop the eruption before the symptoms of tumor. The time interval between the eruption of erythema gyratum repens and the detection of the tumor can range from simultaneous presentation to up to 6 years after the rash. Erythema gyratum repens also has occurred up to 7 months after the detection of malignancy.

Erythema gyratum repens is associated with some nonneoplastic conditions, such as pulmonary tuberculosis, cryptogenic organizing pneumonia,[12]  lupus erythematosus, CREST (calcinosis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) syndrome, virginal breast hypertrophy, pityriasis rubra pilaris,[13, 14, 15]  psoriasis,[16] and as a drug reaction to azathioprine in a patient with type I autoimmune hepatitis.[17] In a few patients, no associated conditions exist.[18]

Epidemiology

Frequency

Erythema gyratum repens is believed to be rare. A clinical review in 1992 by Boyd cited 49 patients reported in the literature.[19] A current literature search yielded a handful of additional case reports.

Race

Erythema gyratum repens reportedly occurs predominantly in white persons.

Sex

Male-to-female ratio is 2:1.

Age

Erythema gyratum repens usually occurs in patients older than 40 years, with a mean age of 63 years, but it has been reported to occur from age 16-77 years.[20]

Prognosis

The prognosis for erythema gyratum repens depends on the underlying illness. In most patients, symptoms disappear with the resolution of underlying disease. No specific complications are associated with the skin manifestations of erythema gyratum repens, and the condition alone does not lead to death. Symptoms include intense pruritus, and morbidity and mortality may occur related to the underlying condition.

History

The appearance of erythema gyratum repens often precedes the detection of malignancy. The skin eruption is present an average of 9 months prior to the diagnosis of malignancy, with a range of 1-72 months. In a minority of patients, erythema gyratum repens occurred simultaneously with, or up to 9 months after, the detection of the neoplasm.

Cases of pityriasis rubra pilaris have been noted to transform to an eruption of erythema gyratum repens upon resolution of the primary disease, without any evidence of internal malignancy.[15, 21, 22, 23]

Physical Examination

Erythema gyratum repens has distinctive dermatologic manifestations characterized by the following[24] :

See the image below.



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Erythema gyratum repens. Courtesy of Jeffrey P. Callen, MD.

Associated findings with erythema gyratum repens include ichthyosis and palmoplantar hyperkeratosis.

 



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Erythema gyratum repens. The characteristic wood-grain pattern is composed of annular, erythematous concentric bands lined by a trailing edge of scale....

Laboratory Studies

A basic workup is warranted when approaching a patient with erythema gyratum repens to search for common or clinically suspected malignancies. This includes the following:

Imaging Studies

Examine patients with erythema gyratum repens for common or clinically suspected malignancies. This workup can include chest radiography and CT scanning of the abdomen and pelvis.

Procedures

Necessary procedures may include the following:

Histologic Findings

The primary finding is mild spongiosis, focal parakeratosis, and a superficial perivascular lymphohistiocytic infiltrate that also may include eosinophils and melanophages. Exocytosis of neutrophils and eosinophils may be observed. Direct immunofluorescence tests sometimes reveal C3, C4, and immunoglobulin G at the basement membrane zone.[32]

Medical Care

Topical steroids are of no benefit in erythema gyratum repens. Corticosteroid injections may improve the pruritus but reportedly do not change the appearance of the skin eruption. Improvement of erythema gyratum repens often occurs with successful treatment of the underlying cancer.

Long-Term Monitoring

Physicians could consider regular screenings in patients with idiopathic eruptions of erythema gyratum repens to address possible development of malignancy as they appear long after the cutaneous eruption.[18] However, given various reports of nonparaneoplastic cases, patients should undergo age-based cancer screening per national guidelines, with symptom-based malignancy evaluations only as clinically indicated until more data are available.[15]

Author

Nicole Ufkes, Medical University of South Carolina College of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Dirk M Elston, MD, Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Disclosure: Nothing to disclose.

Specialty Editors

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD, Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Disclosure: Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Allergen; Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

Dirk M Elston, MD, Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Carrie L Kovarik, MD, Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine

Disclosure: Nothing to disclose.

H Harris Reynolds, MD, Medical Department Head and Flight Surgeon, Training Air Wing SIX, Naval Air Station; Lieutenant, Medical Corps, United States Navy

Disclosure: Nothing to disclose.

Karen D Allen, MD, Consulting Dermatologist, Private Practice

Disclosure: Nothing to disclose.

Raul Del Rosario, MD, Consulting Staff, Dermatopathology, Mission Hospital at Laguna Beach

Disclosure: Nothing to disclose.

Suzanne Kaneshiro, MD, Private Practice, Honolulu, HI

Disclosure: Nothing to disclose.

References

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  18. Tchernev G, Chokoeva AA, Vidolova NG, Ivanova B, Mangarov H, Nikolovska D, et al. Erythema gyratum repens: a pathogenetic mystery and therapeutic challenge. Wien Med Wochenschr. 2017 Apr. 167 (5-6):117-119. [View Abstract]
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  26. Spierings NM, Natkunarajah J. Leucocytoclastic vasculitis presenting as an erythema gyratum repens-like eruption. Clin Exp Dermatol. 2016 Apr. 41 (3):320-2. [View Abstract]
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  28. Jouary T, Lalanne N, Stanislas S, Vergier B, Delaunay M, Taieb A. Erythema gyratum repens-like eruption in mycosis fungoides: is dermatophyte superinfection underdiagnosed in cutaneous T-cell lymphomas?. J Eur Acad Dermatol Venereol. 2008 Nov. 22(10):1276-8. [View Abstract]
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  31. Hauschild A, Swensson O, Christophers E. Paraneoplastic bullous pemphigoid resembling erythema gyratum repens. Br J Dermatol. 1999 Mar. 140(3):550-2. [View Abstract]
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Erythema gyratum repens. The characteristic wood-grain pattern is composed of annular, erythematous concentric bands lined by a trailing edge of scale.

Erythema gyratum repens. Courtesy of Jeffrey P. Callen, MD.

Erythema gyratum repens. The characteristic wood-grain pattern is composed of annular, erythematous concentric bands lined by a trailing edge of scale.

Erythema gyratum repens. Courtesy of Jeffrey P. Callen, MD.

Erythema gyratum repens. The characteristic wood-grain pattern is composed of annular, erythematous concentric bands lined by a trailing edge of scale.