Annually Recurring Erythema Annulare Centrifugum: A New Case Series with Review of the Literature

Abstract

Annually recurring erythema annulare centrifugum (AR-EAC) is a rare variant, characterized by typical annular plaques recurring in the same period of the year. We describe 5 new cases and present a review of the literature. Patients were 3 females and 2 males with an age range of 25–55 years. Multiple annular plaques were located at the thighs in 4 patients and the neck in one patient. In 1 patient, a single lesion was present. Plaques were recurring in summer in 3 cases; in 1 case, in spring; and another patient, in winter since 3–4 years. Lesions were self-healing in few days or weeks. Histologically, the epidermis presented mild acanthosis with patchy spongiosis, slight parakeratosis, and mild exocytosis. There was a perivascular lympho-histiocytic infiltrate of variable intensity in the superficial dermis, with occasional eosinophils. In 1 case, the inflammatory infiltrate reached the deep dermis. Mucin deposition was absent. Phenotyping studies in 1 case revealed a predominance of T cells, with a small B-cell component. Moreover, a moderate number of CD123<sup>+</sup> plasmacytoid dendritic cells and CD1a<sup>+</sup> dendritic cells were noted. Fourteen cases of AR-EAC have been published previously. Collectively, patients’ age ranged from 16 to 83 years, with a mean age of 47 years and a disease duration of 1–30 years. Lesions affected more frequently extremities and recurred most commonly in summer. Patients were all in good general health. Topical corticosteroids were the mainstay of treatment. AR-EAC is a benign disorder, the nature of which remains enigmatic.

Uncommon Non-Infectious Annular Dermatoses

“…To make the topic even more intriguing, we recently reported a case of AR-EAC deep type with periadnexal involvement. [ 9 ] The immunophenotyping studies revealed a moderate number of CD123 + plasmacytoid dendritic cells, which are well documented in Jessner-Kanof and LET but not in EAC, with the caveat that only one case has been evaluated. [ 41 42 ] In conclusion, there is significant clinical and histological overlap between PMAE, Jessner-Kanof, LET, and EAC deep type.…”

“…[ 6 7 8 ] Only 14 cases have been described in the literature and have been recently reviewed. [ 9 ] Patient age ranged from 16 to 83 years, with a mean age of 47 years and a disease duration of 1–30 years. In most cases, AR-EAC has been observed in females, with an M:F = 1:2.4.…”

“…AR-EAC has not been associated with any underlying disorder or other causative factors. [ 7 8 9 ] Lesions tend to resolve spontaneously without leaving skin marks. The treatment is based on topical corticosteroids.…”

Annually recurring erythema annulare centrifugum – a rare entity

“…Annually recurring erythema annulare centrifugum is a very rarely reported variant of EAC which clinically and histopathologically simulates classic superficial EAC but recurs periodically in each year 1,2 . It has a female preponderance with a peak age of onset between 40 to 50 years 1,2 , this can range from 16 to 80 years. Even though this variant is idiopathic, a full diagnostic workup is mandatory to exclude any underlying disorder.…”

“…Annually recurring erythema anulare centrifugum is a very rarely reported peculiar variant of EAC which has identical clinical and histopathological features to classic EAC 1 . Up to now very few similar cases are reported in the literature.…”