Atypical skin and mucosal lesions with renal involvement in ANCA vasculitis - a case report.

Abstract

Systemic vasculitis is a group of connective tissue diseases in which blood vessel wall is destroyed due to inflammatory process. It is a very heterogeneous clinical entity in which symptoms depend on the caliber of vessels and organs involved. Symptoms are often associated with changes in the vasculature of the skin, kidneys and the upper or lower respiratory tract. The onset of the disease can be very clandestine or highly nonspecific, which makes a quick and accurate diagnosis extremely difficult. A definitive diagnosis is sometimes not made until patient present with severe symptoms, including alveolar hemorrhage with respiratory failure or acute kidney injury, requiring urgent hospitalization. Skin and mucosal lesions are known to be related with the severity of organ involvement and can be specific for various types of vasculitis. Since in most cases they occur simultaneously or after the onset of general symptoms, they can help to make the right diagnosis. Presented study is a brief case report of a patient with atypical skin and mucosal lesions, admitted due to acute kidney injury, anemia, and rhabdomyolysis, in whom anti-neutrophil cytoplasmic antibodies (ANCA) vasculitis was diagnosed. Systemic vasculitis is a rare group of diseases that can be life-threatening. For this reason, it is of utmost importance to make a proper diagnosis, especially in patients with unclear, difficult to treat, recurrent and atypical symptoms. Delayed diagnosis and targeted treatment may lead to advanced and irreversible organ damage. Vasculitis should be taken under consideration in patients with atypical skin and mucosal lesions.