Erythema annulare centrifugum: report of a case with neonatal onset

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 sclerodermic 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.