{'fraction_non_alphanumeric': 0.02669149596523898, 'fraction_numerical': 0.010242085661080074, 'mean_word_length': 5.484909456740443, 'pattern_counts': {'":': 0, '<': 0, '<?xml version=': 0, '>': 0, 'https://': 0, 'lorem ipsum': 0, 'www.': 0, 'xml': 0}, 'pii_count': 0, 'substrings_counts': 0, 'word_list_counts': {'cursed_substrings.json': 0, 'profanity_word_list.json': 0, 'sexual_word_list.json': 0, 'zh_pornsignals.json': 0}}
Remission of incapacitating acute cutaneous lupus erythematosus in a patient with systemic lupus erythematosus by B cell-depletive therapy.

BcellYdepleting therapy has been successfully used for rheumatoid arthritis and could be a novel strategy for numerous other autoimmune diseases. We report a patient with incapacitating lupus skin disease refractory to conventional therapy. Rituximab (anti-CD20) treatment resulted in a remarkable improvement of the skin lesions and quality of life. Systemic lupus erythematosus (SLE) was diagnosed in a 30-year-old woman at age 13 years based on arthritis, skin lesions, serositis, Raynaud phenomenon, antinuclear antibody, and antiYdouble-stranded DNA antibodies. Three years later, she had a biopsy-proven diagnosis of SLE nephritis. The kidney function remained normal while treated with azathioprine, hydroxychloroquine, and corticosteroids. During this treatment, at age 28 years, skin lesions on her hands, feet, face, neck, arms, chest, and abdomen worsened, and the dosage of prednisolone was increased to 60 mg without improvement. Clinically, the rash on the trunk, arms, hands, and fingers represented the disseminated maculopapular variant of acute cutaneous lupus erythematosus (ACLE). Annular-polycyclic or papulosquamous (psoriasiform) skin lesions as in subacute lupus erythematosus or scarring lesions as in discoid lupus erythematosus were not observed. Lesions of the hands were incapacitating as the patient could not use them normally because of pain upon touch. A skin biopsy confirmed the diagnosis of cutaneous lupus and showed granular depositions of IgG and complement along the basal membrane by direct immunofluorescence. Based on positive reports on the chimeric anti-CD20 monoclonal antibody rituximab for the treatment of SLE, we treated her with 2 intravenous infusions of rituximab (1000 mg) with an interval of 2 weeks, concomitant with methylprednisolone 100 mg and clemastine 2 mg as premedication. This had a dramatic positive effect on her skin lesions (Fig.). Three months after treatment, all skin lesions were in complete remission, and the prednisolone dosage could be tapered to 7.5 mg once daily. The antiYdouble-stranded DNA antibody levels, shown by Farr assay, decreased from 157 IE/mL before treatment to 38 IE/mL 10 months after treatment. The skin lesions relapsed 9 months later and again responded spectacularly after another course of rituximab. Skin manifestations represent the second most common symptoms of SLE and often require systemic treatment. Antimalarials are the drugs of first choice, but the effects are not always sufficient. Various immunosuppressive drugs have been used in resistant cases, including azathioprine, methotrexate, mycophenolate mofetil, and thalidomide, often combined with corticosteroids. Alleviating refractory and incapacitating skin lesions, if present, is often the most important issue for the patient. The efficacy of the B cellYdepleting antibody rituximab is illustrated by this case. Consistent with its known long-lasting pharmacokinetic effects, we observed clinical improvement of previously therapy-resistant skin disease for a period of 9 months after a single treatment course.