Erythema Annulare Centrifugum

Updated: Jul 18, 2017
  • Author: Marisel Peralta-Abejo, MD, DPDS; Chief Editor: William D James, MD  more...
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Overview

Background

Erythema annulare centrifugum (EAC) is classified as one of the figurate or gyrate erythemas. First described by Darier in 1916, it is characterized by a scaling or nonscaling, nonpruritic, annular or arcuate, erythematous eruption. It tends to spread peripherally while clearing centrally. Histologically, an intense lymphohistiocytic cuffing occurs about the superficial and deep dermal vessels without epidermal involvement. The etiology is uncertain, but it may be due to a hypersensitivity to malignancy, infection, drugs, or chemicals, or it may be idiopathic.

Controversy exists in the classification of the gyrate erythemas, and the literature is wrought with ambiguity and contradictions. Since its initial description in 1916, the term erythema annulare centrifugum has grown to include several histologic and clinical variants. Ackerman, and later Bressler and Jones, [1] suggested a classification in which only 2 types of gyrate erythema are considered: superficial (pruritic, scaling) and deep (nonpruritic, nonscaling). The original description of EAC was of the latter type. However, the superficial type is more commonly seen with its characteristic trailing scale behind an advancing, erythematous border.

In this article, EAC is considered to include all the gyrate erythemas, except for erythema marginatum rheumaticum, erythema chronicum migrans, and erythema gyratum repens. When taken in this broad sense, EAC can be scaly or nonscaly, pruritic or nonpruritic, and rarely vesicular.

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Pathophysiology

The pathogenesis of erythema annulare centrifugum (EAC) is unknown, but it is probably due to a hypersensitivity reaction to a variety of agents, including drugs, arthropod bites, infections (bacterial, mycobacterial, viral, fungal, filarial), ingestion (blue cheese Penicillium), and malignancy. Injections of Trichophyton, Candida, tuberculin, and tumor extracts have been reported to induce EAC, supporting a type IV hypersensitivity reaction as at least one mechanism for its development. Another purported mechanism in the pathogenesis of EAC is that of a Th1-mediated reaction with elevated levels of tumor necrosis factor-alpha and associated proinflammatory cytokines. Minni and Sarro [2] reported response to (and relapse following cessation of) etanercept in a 57-year-old white man as evidence supporting this theory.

Other cases of EAC have been found in association with an underlying systemic or infectious disease (eg, liver disease, [3] Sjögren syndrome, systemic lupus erythematosus, Graves disease, [4] hypereosinophilic syndrome, [5] appendicitis [6] ), herpes zoster, [7] chronic lymphocytic leukemia, and HIV disease. Drugs reported to cause EAC include finasteride, piroxicam, hydroxychloroquine, amitriptyline, and spironolactone. Still other cases have been attributable to a familial form. However, in most cases, no underlying cause can be found. One study of 24 cases of EAC with special reference to its association with an underlying disease found no increased incidence of systemic disease, malignancy, or infection. [8] In another study of 113 cases of gyrate erythemas, 7 cases (none of which was erythema gyratum repens) were associated with internal malignancy compared with 6 cases in the control group.

Hypotheses about the mechanism of annularity focus on the interaction between mediators of inflammation and ground substance as foreign antigens diffuse through the skin.

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Epidemiology

Defining the incidence and the prevalence of erythema annulare centrifugum (EAC) is difficult because the literature mostly consists of case reports and brief reviews. In a review of 24 cases in England, the incidence was reported to be approximately 1 case per 100,000 population per year in a catchment area of 500,000 people.

Race

Whether any racial predilection exists for erythema annulare centrifugum (EAC) is not known.

Sex

No bias for either sex is apparent for erythema annulare centrifugum (EAC).

Age

Erythema annulare centrifugum (EAC) has been reported in patients from infancy to the ninth decade of life.

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Prognosis

The prognosis for erythema annulare centrifugum (EAC) is excellent, except when associated with an underlying malignancy and other systemic disease. The mean duration of erythema annulare centrifugum (EAC) is 11 months. However, the course has ranged from 4-6 weeks to 34 years (recurrent attacks). Most cases require no treatment and resolve spontaneously. Others have been reported in association with malignancy, with the eruptions responding to treatment of the underlying neoplasm. In those cases, the prognosis is affected by the underlying malignancy. Annually recurring erythema annulare centrifugum (AR EAC) was reported in 2015. [9] Treatment of the underlying disorder is effective. If associated with pregnancy, it resolves after delivery.

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