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Case ReportsAbstract
Eosinophilic annular erythema (EAE) is a rare, superficial eosinophilic dermatosis characterized by large annular plaques with intense peripheral erythema and central pigmentation, histologically defined by a perivascular lymphocytic infiltrate rich in eosinophils. We report a unique case of EAE in a 57-year-old male with severe seronegative rheumatoid arthritis (RA) and profoundly elevated serum IgE (>50,000 IU/mL). The patient presented with widespread non-scaly annular papules and plaques involving the trunk, face, and extremities. Biopsy of a lesion on the left breast showed perivascular and periadnexal infiltrates rich in eosinophils. Direct immunofluorescence was negative. Histopathology raised a broad differential such as arthropod assault, hypereosinophilic syndrome, and drug eruption; however, clinically in our case, EAE and Wells syndrome were considered plausible. While Wells syndrome may herald idiopathic hypereosinophilic syndrome (HES), our patient had normal eosinophil counts but marked IgE elevation. Although HES has been reported in autoimmune diseases, including RA, its link to seronegative RA is rare. Extremely elevated IgE may indicate immune dysregulation or an inborn error of immunity. Literature suggests possible, though inconsistent, associations between IgE elevation and autoimmune conditions such as RA. The coexistence of EAE and seronegative RA with markedly elevated IgE raises the possibility of an unrecognized systemic immune disorder. Given the diagnostic complexity, evaluation by dermatology, dermatopathology, rheumatology, allergy/immunology, and consideration of genetic testing is warranted. This case highlights the importance of a multidisciplinary approach in rare dermatoses with systemic implications.