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Background
This peculiar skin rash is characterized by an eruption go annular lesions that enlarge rapidly and usually diappear withing 1-2 weeks. Through the years, the term has been applied to a bewhildering number of skin conditions and may in fact be mistaken for many diseases, notably subacute cutaneous lupus erythematosus. Some experts believe this term should only be applied to the superficial and not the deep variants.
OUTLINE
Epidemiology Disease Associations Gross Appearance and Clinical Variants Histopathological Features and Variants Commonly Used Terms Internet Links
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Figurate erythema INCIDENCE/PREVALENCE Uncommon AGE Usually adults
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL
Clinicopathologic analysis of 66 cases of erythema annulare centrifugum.
Kim KJ, Chang SE, Choi JH, Sung KJ, Moon KC, Koh JK.
Department of Dermatology, Asan Medical Center, University of Ulsan, Seoul, Korea.
J Dermatol. 2002 Feb;29(2):61-7 Abstract quote.
Erythema annulare centrifugum (EAC) denotes a group of eruptions characterized by slowly migrating annular or configurate erythematous lesions. It can be classified histopathologically as the deep or superficial type. Although there are many case reports of EAC associated with various underlying conditions, no recent clinicopathologic studies exist.
The purpose of this study was aimed at analyzing the clinical and histopathologic features of EAC. Sixty-six patients who have been diagnosed as EAC by clinical and histopathological examination were collected. The medical records and skin biopsy specimens of these patients were reviewed retrospectively. There were 24 male patients and 42 female ones. The mean age was 39.7 years, and the mean durtion of the disease was 2.8 years. The lower extremities, particularly the thigh, were the most frequently involved locations. The most common clinical manifestation was large (>1 cm), scaly, erythematous, indurated plaques. Forty-eight patients (72%) had combined diseases including cutaneous fungal infection (48%), such as tinea pedis, other skin diseases (18%), internal malignancies (13%), and other systemic diseases (21%). By histopathologic examination, 33 of 42 patients (78%) were identified as superficial type, and 9 patients (22%) were deep type.
Therapeutic trials with systemic or topical corticosteroid and oral antihistamine did not affect the chronic and recurrent course of these patients. EAC is a chronic and recurrent disease despite treatment. EAC is thought to be highly associated with internal disease as well as with superificial fungal infection. However, it was difficult to prove a causal association. The recognition and exact diagnosis of EAC is important, because it may be a quite stressful condition and lead to unnecessary over-treatments.
Erythema annulare centrifugum.
Bressler GS, Jones RE Jr.
J Am Acad Dermatol. 1981 May;4(5):597-602. Abstract quote
If one reviews the literature on the subject of erythema annulare centrifugum, it becomes quite obvious there is considerable confusion about the clinical presentation and histopathologic findings.
This confusion is exemplified by the various quotes from dermatologic texts and scientific publications. Darier, who originally described the disease, described an annular, indurated, erythematous lesion without a scale that histologically was characterized by a superficial and deep lymphohistiocytic infiltrate and normal epidermis. Ackerman suggested that there are two types of gyrate erythema, a superficial type showing a scale, and the deep type as described by Darier.
After reviewing the literature and studying patients with gyrate erythemas, it seems that there are two distinct types best termed the superficial and deep forms of gyrate erythema. Dermatologists use the term erythema annulare centrifugum to denote both of these forms. Perhaps that term should be discarded.VARIANTS NEONATAL
Erythema annulare centrifugum: report of a case with neonatal onset.
Bottoni U, Innocenzi D, Bonaccorsi P, Carlesimo M, Faina P, Richetta A, Cugini P, Calvieri S.
Department of Dermatology, University of Rome La Sapienza, Italy.
J Eur Acad Dermatol Venereol. 2002 Sep;16(5):500-3. Abstract quote
Darier's erythema annulare centrifugum (EAC) is often associated with infectious, autoimmune or neoplastic disease, nevertheless, most cases of EAC remain unexplained. We report a case of EAC with neonatal onset and a clinical course of over 20 years.
The patient presented from the 6th day of life with erythematous papules that enlarged centrifugally to form figurate, annular or policyclic plaques involving the entire cutaneous surface. The clinical picture had a chronic course, disappearing only during unrelated febrile episodes. Histologic examination revealed a normal epidermis and a dense perivascular lymphohistiocytic infiltrate and numerous eosinophils in the superficial and reticular dermis. Today the patient is 24 years old and his lesions are still unchanged. Recently, clinical evaluation revealed a diffuse sderodermic induration. A second biopsy showed a diffuse thickening of collagen fibers in the dermis.
To our knowledge, this is the second report of EAC with neonatal onset and such a long and peculiar clinical course.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL
Erythema annulare centrifugum: results of a clinicopathologic study of 73 patients.
Weyers W, Diaz-Cascajo C, Weyers I.
Center for Dermatopathology, Freiburg, Germany.
Am J Dermatopathol. 2003 Dec;25(6):451-62. Abstract quote
Erythema annulare centrifugum is classified generally into a superficial and a deep type. Whether those types are variants of the same process or unrelated to one another, and whether they represent non-specific patterns or specific clinico-pathologic entities, is controversial.
To answer those questions, we analyzed 82 biopsy specimens from 73 patients with a clinical and histopathologic diagnosis of erythema annulare centrifugum, gyrate erythema, or figurate erythema regarding a variety of clinical and histopathologic findings.
We found substantial differences between cases with a wholly superficial type and cases with a superficial and deep infiltrate. Clinically, a collarette of scales was seen only in the superficial type. Histopathologically, some findings were much more common in the superficial type (eg, spongiosis, parakeratosis, crusts, edema of the papillary dermis, epidermal hyperplasia) and others in the deep type (eg, sleeve-like arrangement of the infiltrate, melanophages, subtle vacuolar changes at the dermo-epidermal junction, individual necrotic keratinocytes). Whereas cases of the superficial type could be distinguished from differential diagnoses by a variety of clinical and histopathologic findings, most cases of the deep type showed subtle signs of lupus erythematosus. Neither type was associated consistently with any other systemic disease. Because the superficial and the deep type of erythema annulare centrifugum seem to be unrelated to one another, they should not be referred to by the same name.
We believe that the term should be reserved for the superficial type because the latter seems to be a specific clinico-pathologic entity. By contrast, most cases of the deep type seem to be annular examples of tumid lupus erythematosus and should be diagnosed that way. If findings militate against the diagnosis of lupus erythematosus, we suggest using a descriptive term that signals non-specificity-namely, deep figurate erythema.Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Eight Edition. Mosby 1996.
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Robbins Pathologic Basis of Disease. Sixth Edition. WB Saunders 1999.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
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Last Updated 12/15/2003
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