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Polyarteritis nodosa (PAN) is a rare systemic necrotizing vasculitis that can lead to the formation of refractory lower limb ulcers requiring amputation. The standard treatment for severe PAN involves combination therapy with steroids and cyclophosphamide; however, some cases prove to be challenging. Recently, case reports have described the use of biological agents for PAN treatment.

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The deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive disorder caused by loss of function mutations in the ADA2 gene (previously the CECR1 gene) on chromosome 22q11. The clinical spectrum of the disease is remarkably broad, and its presentations mimic features of polyarteritis nodosa, such as livedoid rash, hematological abnormalities (e.g.

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Polyarteritis nodosa (PAN) is a rare systemic necrotizing vasculitis preferentially targeting medium-sized arteries. PAN has two clinical entities: systemic PAN (sPAN) and cutaneous PAN (cPAN). cPAN is a skin-limited vasculitis, while ulcerative cPAN often predicts a higher risk of recurrence and a worse prognosis.

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  • Multiple sclerosis (MS) is a chronic disease that severely affects quality of life and can co-occur with various other conditions, including primary vasculitis, which complicates diagnosis and treatment.
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