Treatment of
skin manifestations in
systemic lupus erythematosus (SLE),
systemic sclerosis (SSc), and
dermatomyositis (DM) is based on the results of only few randomized controlled trials. The first-line treatment for disfiguring and widespread cutaneous involvement in SLE is
antimalarials, but some patients are
therapy resistant. Recently, the
monoclonal antibody belimumab was approved for SLE as an adjunct
therapy for patients with
autoantibody-positive disease who despite standard
therapy show high disease activity, intolerance of other treatments, or an unacceptably high need for
corticosteroids. However, a validated skin score has not been used to confirm the efficacy of
belimumab on mucocutaneous manifestations. In SSc, another multi-systemic progressive disease, involvement of the lung, kidney, and the heart is frequently treated with
corticosteroids and immunosuppressives, but therapeutic modalities for cutaneous lesions, such as skin
sclerosis and
digital ulcers, are limited. In the past years, treatment with the
endothelin-receptor antagonist bosentan has been proven to reduce the occurrence of new
digital ulcers in SSc patients but has no or limited effect on healing of
digital ulcers. DM is an idiopathic
autoimmune disease characterized by
inflammation of the muscles and skin, which is treated with immunosuppressives.
Corticosteroids are the first-line treatment for muscle involvement in DM, but skin lesions often flare by reduction or discontinuation. In summary, there is a high unmet need for new therapeutic strategies focusing on skin involvement in systemic
autoimmune diseases. Therefore, innovative designs of randomized controlled trials with validated skin scores are warranted to develop new therapeutic strategies for patients with cutaneous manifestations.