Connective tissue diseases (CTDs) are a group of chronic autoimmune conditions characterized by variable involvement of connective tissues, including the skin. Cutaneous manifestations are often observed in the course of lupus erythematosus, dermatomyositis and systemic sclerosis, most commonly as the presenting symptoms. In this paper, we review specific and non-specific symptoms and signs associated with skin involvement in these important CTDs, correlating them with systemic manifestations and laboratory findings, aiming to improve diagnosis accuracy. In the light of evidence-based practice, recommendations for individual assessment and relevant nuances to the differential diagnosis are critically appraised in order to improve diagnosis accuracy and consequently the treatment success.