A Clinical Case of an Adult Patient with Dermatomyositis, Characterized by Marked Calcinosis with “Ulcerations” and Positive Anti-NXP-2 (anti-MJ) Autoantibodies

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.


Desislava Kalinova, Ekaterina Ivanova-Todorova, Dobroslav Kyurkchiev, Rasho Rashkov and Valentina Reshkova

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