Case Report
Desislava Kalinova, Ekaterina
Abstract
Autoimmune myositis is a syndrome, characterized by involvement of the cellular and humoral immune systems in skeletal muscle pathology, response to immunotherapies, and the presence of autoantibodies (Abs) (myositis-specific and myositis-associated Abs) in the serum of some patients. Myositis-specific Abs are found almost exclusively in patients with myositis and are associated with definite subsets of myositis characterized by distinct clinical features. It has been suggested that myositis-specific Abs may serve as a diagnostic serologic marker for different specific subset. A new Abs, called anti-NXP-2 Abs, has been identified in juvenile dermatomyositis (JDM) patients, and it was associated with severe muscle weakness, polyarthritis, joint contractures, and interstial vasculitis. Anti-NXP-2 Abs are also found in adult polymyositis/dermatomyositis (PM/ DM). We present a clinical case of 39-years old female patient with distinct subset of dermatomyositis with positive anti-NXP-2 Abs, clinically characterized by marked calcinosis with "ulcerations", typical skin lesions (heliotropic rash, Gottron papules), proximal muscle weakness, without internal organ damage.