VEXAS syndrome initially presenting as relapsing polychondritis and progressing into neutrophilic dermatosis with systemic symptoms over a 5‐year period.

The article discusses a case of VEXAS syndrome in a Japanese patient who initially presented with relapsing polychondritis in the earlobes and later developed neutrophilic dermatosis with systemic symptoms over a 5-year period. The patient's condition progressed rapidly, leading to a critical stage of the disease with leukocytoclastic vasculitis and lung opacities. VEXAS syndrome is a newly established autoinflammatory disorder driven by somatic variants in UBA1, with skin manifestations such as red or violaceous papules and plaques. The study highlights the importance of recognizing specific symptoms like auricular chondritis, periodic fever, and neutrophilic dermatosis for diagnosing VEXAS syndrome, which can have a severe clinical course and high mortality rates. [Extracted from the article]