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Abstract Cutaneous lupus erythematosus is an autoimmune disease of varied clinical expression, which may present as an exclusively cutaneous disease or be one of the multiple manifestations of systemic lupus erythematosus. Its classification includes acute, subacute, intermittent, chronic and bullous subtypes, which are usually identified based on clinical features and histopathological and laboratory findings. Other non-specific cutaneous manifestations may be associated with systemic lupus erythematosus and are usually related to disease activity. Environmental, genetic and immunological factors play a role in the pathogenesis of skin lesions in lupus erythematosus. Recently, considerable progress has been made in elucidating the mechanisms involved in their development, which allows for foreseeing future targets for more effective treatments. This review proposes to discuss the main etiopathogenic, clinical, diagnostic and therapeutic aspects of cutaneous lupus erythematosus, aiming to update internists and specialists from different areas.
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Objective To investigate clinical and pathological features of lupus erythematosus profundus (LEP)of the scalp in children.Methods A retrospective study was carried out on 5 children with LEP.The clinical and histopathological features,treatment and prognosis of LEP were analyzed.Results The 5 children with LEP included 2 boys and 3 girls with a median age at onset of 5 months (range,2-38 months) and a median clinical course of 15 months (range,4-72 months).Clinically,the patients presented with arc-shaped or circular purple atrophic plaques on the scalp complicated by alopecia.The occipitalia and tempora were the most commonly involved sites.Antinuclear antibodies (ANA) and extractable nuclear antigens (ENAs) were negative in all the patients.Main histopathological changes were hyaline degeneration of the fat,mucin deposition and local aggregation of lymphocytes in fat lobules.Of the 5 patients,2 were treated with oral prednisone (1.5-2 mg/kg/day),1 with oral hydroxychloroquine (5 mg/kg/day),1 with oral prednisone (1.5 mg/kg/day) combined with hydroxychloroquine (5 mg/kg/day),and another 1 with topical halometasone cream and 0.03％ tacrolimus ointment.Lesions were remissed after 2-3 months of treatment,and subsided with growth of new hairs after 6 months.No recurrence was observed during a 1.5-year follow-up.Conclusion Prednisone and hydroxychloroquine are markedly effective for LEP,and pediatric patients with LEP may be treated by topical highpotency glucocorticoids and calcineurin inhibitors.
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SUMMARY A 36-year-old woman had an 8-year history of systemic lupus erythematosus (SLE) and was being treated with 10 mg/d of prednisone.She presented with a 6-month history of intermittent fever and multiple painful multi skin erythematous macules in her button, hips and extremities that had slowly en-larged to 8 cm ×4 cm in diameter.The lesions started as painful erythematous macules, which eventually ulcerated and scared.Laboratory tests showed leukopenia, protenuria, positive anti-double strand DNA and hypocomplementemia.Cultures of the bottom ulcer were E.Coli, fugus and Tuberculous mycobacteria were both negative.Biopsy was performed and revealed necrosis of epidermis, thrombus and cellulose de-generation in epidermis with neutrophils karyorrhexis and vasculitis.Her SLE was active, so she was pre-scribed antibiotics for 2 weeks and prednisone was added to 60 mg/d for a month.However her skin ul-cers did not relieve.When prednisone was added to 120 mg/d with combination therapy of cyclophospha-mide and hydroxychloroquine, her skin ulcer cicatrized gradually.