Subacute cutaneous lupus erythematosus with pityriasis-like cutaneous manifestations

A 57-year-old woman was seen in our department in September 1999. At the ti me of our observation, she complained of fever and malaise and presented ma ny erythematous and squamous lesions on sun-exposed areas. On the upper third of the back (Figs 1, 2), she presented a wide patch with irregular borders, due to many coalescing small purplish-red lesions. Each lesion was covered with branny scales and, on palpation, revealed a variab le degree of infiltration. Few areas of uninvolved skin could be seen insid e the main patch. Beyond this main patch, small individual scattered lesion s with a net-like distribution were detected. Similar lesions were present on the neck, chest, and arms. Her face showed diffuse erythema with fine scaling, except for the nasolabial folds which w ere unaffected. Telangiectasias were present on her face, trunk, limbs, and periungual areas. The patient stated that such lesions had developed after sun exposure. In 1990, a diagnosis of systemic lupus erythematosus (SLE) was made accordi ng to the presence of five American Rheumatism Association (ARA) criteria: photosensitivity, malar eruption, diffuse arthralgias (associated with swel ling of the finger joints), mild renal involvement with proteinuria, and le ukopenia. Since then she has also presented anti-Ro/ SSA antibodies, hypert ension, and Raynaud's phenomenon. A histologic study performed on the skin of the back showed a moderate epid ermal atrophy with a slight hyperorthokeratosis, a pronounced hydropic dege neration of the basal cell layer, an intense band-like and perivascular lym phocytic infiltrate, and a moderate diffuse edema of the upper dermis (Fig. 3). Laboratory analysis revealed positive anti-dsDNA (titer 1 :160) and anti-Ro /SSA antibodies, iron-deficiency anemia, leukopenia (3000/mm(3)), microhema turia, and persistent proteinuria (0.7 g/day). Hypocomplementemia (C3, 47 m g/dL; C4, 10.4 mg/dL) and increased immunoglobulin G (IgG) serum levels wer e found, while circulating immune complexes could not be demonstrated. The creatinine clearance was found to be decreased (53 mL/min). Chest X-ray and echocardiography were negative, but a holter examination re vealed an abnormal ventricular rhythm. Direct immunofluorescence performed on lesional skin of the back revealed a granular deposition of IgM, C3 at the dermal-epidermal junction (DEJ), a p erivascular deposition of C3, and dust-like particles of IgG in the epiderm is. We confirmed the previous diagnosis of SLE and classified the unusual clini cal manifestations as pityriasis-like subacute cutaneous lupus erythematosu s (SCLE). The patient was treated with systemic and local steroids (prednisone, 0.5 m g/kg/day) with a marked improvement of the cutaneous manifestations.