Unilateral Periorbital Edema and Erythema: A Rare Presentation of Chronic Cutaneous Lupus Erythematosus

Abstract

onnective tissue autoimmune diseases such as systemic lupus erythematosus (SLE), dermatomyositis or scleroderma often prominently affect the skin. Specific or nonspecific cutaneous lesions in lupus erythematosus are very common. It is well known that the lupus erythematosus rash shows variable features and is categorised into acute, subacute and chronic varieties according to the clinical and histopathological appearances and duration of symptoms. A proportion of cutaneous lupus erythematosus (CLE) cases can involve only skin or present as part of the symptoms of SLE. 1 Immunologically a positive antinuclear antibody (ANA) is found in 95% of the patients presenting with malar rash with acute cutaneous lupus eritematosus. Antinuclear antibody is positive in 70-80% of the patients showing annular and papulosquamous lesions with subacute cutaneous lupus erythematosus. 2 Chronic cutaneous lupus (CCLE) includes different lesions such as discoid lupus eritematosus (DLE), lupus erythematosus profundus,chilblain lupus and lupus tumidus. Serologically, DLE patients have a lower incidence of autoantibodies such as ANA ds-DNA and SS-A (Ro) as compared to other CLE subtypes. 3 Unilateral or bilateral periorbital skin involvement with or without systemic features or other cutaneous findings in lupus patients is a relatively rare clinical presentation of CLE. 4 Turkiye Klinikleri J Case Rep. 2019;27(4):241-4 241 Unilateral Periorbital Edema and Erythema: A Rare Presentation of Chronic Cutaneous Lupus Erythematosus A AB BS S T TR RA AC CT T We present a case of a 55-year-old female with unilateral periorbital edema and erythema over the past one year without systemic features or other skin signs, who had photosensitivity and positive antinuclear antibody and also SS-A 60kD antibody. Treatment with topical corticosteroids had remained unsuccessful along one year. We suspected a clinical picture association with cutaneous lupus erythematosus including photosensitivity,chronic cutaneous lesion finding and positive autoantibodies and observed a fovarable clinical response to systemic antimalarial therapy within a short time. The observation has confirmed our diagnosis. Similar cases were rarely reported in literature. The persistent edema and erythema involving periorbital area can occur as a rare manifestation of chronic cutaneous lupus erythematosus.