Mehr als nur der Schmetterling – ein Leitfaden durch die Vielfalt des kutanen Lupus erythematodes: Pathogenese, Klinik, Therapie

The plethora of manifestations of cutaneous lupus erythematosus (CLE) can occur with or without concomitant systemic lupus erythematosus (SLE) for which regular screening is mandatory. Female sex, genetic predisposition, sun exposure, smoking and some medications serve as risk factors for LE. The most important CLE forms are the acute-cutaneous LE (e.g., butterfly rash, generalized macular–papular rash, enoral), the subacute-cutaneous LE (e.g., annular) and the chronic-cutaneous LE (e.g., scaring discoid lesions, panniculitis, chilblain-LE). Diagnosis is primarily based upon the clinical picture and its histopathology. Results of autoimmune serology and direct immunofluorescence can be helpful. For mild forms of CLE local therapy is often sufficient. If not, hydroxychloroquine is the first choice of systemic therapy in addition to transient systemic steroids. For recalcitrant cases additional therapy with methotrexate, retinoids, dapsone, mycophenolate mofetil, azathioprine, thalidomide, belimumab and rituximab may be used. All CLE therapies are off-label. An update of conventional vaccinations is advisable, if possible, to be performed before starting immunosuppressive therapies. To objectively verify the therapeutic response, the periodic scoring using the RCLASI (Revised CLE Disease Area and Severity Index) is recommended. Preventive sun protection (cream, clothing, travel destinations) is of pivotal importance, since intense UV-exposure can provoke disease flares. LE is no contraindication against a pregnancy. However, pregnancy should not start during a disease flare since it increases the risk for adverse outcomes for the fetus and mother. Steroids, hydroxychloroquine, dapsone and azathioprine serve as suitable therapies during pregnancy.