Viruses have long been held to be of pathogenetic importance in the evolution of autoimmune
connective tissue disease. We describe 7 adults who developed cutaneous
connective tissue disease stigmata in temporal association with recent cytomegalovirus (CMV)
infection but without the classic cytopathic changes of CMV
infection. We examined 7 adults with clinical presentations encompassing
cutaneous vasculitis in 4 and scleroderma in 3. In all 7 patients, there was either
IgM seropositivity for CMV and/or CMV
DNA isolation from peripheral blood. Although no
CMV inclusions were seen, in situ hybridization studies revealed very focal CMV
RNA transcript expression with localization mainly to the endothelium. The patients with
vasculitis treated with
ganciclovir had improvement or resolution of symptoms, whereas only 1 patient with scleroderma received
antiviral therapy, without benefit. Another scleroderma patient responded to
infliximab therapy. Abortive/partial CMV reactivation can be associated with a syndrome complex mimicking and/or triggering a primary immune-based cutaneous microvascular injury syndrome.
Antiviral therapy appears to be of therapeutic value in those cases associated with active necrotizing vasculitic changes. The role of
tumor necrosis factor alpha blockers in scleroderma cases temporally associated with CMV
infection requires further evaluation.