The skin is one of the major organs involved in systemic lupus erythematosus (SLE) and may provide the first sign of the disease. Cutaneous manifestations comprise various specific and nonspecific symptoms that can be isolated or combined and are not amply reflected by currently available scoring systems. From the dermatological viewpoint, three distinct subsets of cutaneous lupus erythematosus can be defined that include chronic cutaneous lupus erythematosus (CCLE), subacute cutaneous lupus erythematosus (SCLE) and acute cutaneous lupus erythematosus. These, in turn, comprise different subsets and result in a heterogenous group of diseases. Based on case history, clinical and laboratory data, as well as organ imaging examinations, they need to be differentiated for prognostic and therapeutic reasons. Whereas CCLE rarely develops into SLE, SCLE and SLE are more closely related. Basic laboratory examinations should be performed on all patients at first diagnosis, whereas specific laboratory examinations will depend on individual data. Altogether, monitoring of lupus erythematosus (LE) patients for internal organ manifestations at any stage of their disease is mandatory. Even in seemingly isolated cutaneous LE, other LE entities should be excluded. Therapy should be initiated in an individually tailored way as either topical external or systemic treatment based on the extent and severity of organ involvement and should be regularly adapted. Thus, both dermatologists and rheumatologists should cooperate to provide optimal patient care.