J Rheum Dis.  2018 Oct;25(4):302-305. 10.4078/jrd.2018.25.4.302.

Successful Treatment of Refractory Cutaneous Polyarteritis Nodosa with Adalimumab

• Ahn E
• So MW

• KMID: 2429715
• DOI: http://doi.org/10.4078/jrd.2018.25.4.302

Abstract

Cutaneous polyarteritis nodosa (CPAN) is a form of necrotizing vasculitis of the medium and small-sized arteries. The condition is limited to the skin and there is a lack of visceral involvement. Treatment with systemic glucocorticoids alone or in combination with azathioprine, methotrexate or cyclophosphamide, depending on the disease severity, has been shown to be effective. This paper reports the clinical case of a 53-year-old female patient with CPAN refractory to treatment with high dose glucocorticoid, methotrexate, azathioprine, and cyclophosphamide, who was treated successfully with anti-tumor necrosis factor-α therapy (adalimumab).

Keyword

Polyarteritis nodosa; Skin; Tumor necrosis factor; Adalimumab

MeSH Terms

Adalimumab*
Arteries
Azathioprine
Cyclophosphamide
Female
Glucocorticoids
Humans
Methotrexate
Middle Aged
Necrosis
Polyarteritis Nodosa*
Skin
Tumor Necrosis Factor-alpha
Vasculitis
Adalimumab
Azathioprine
Cyclophosphamide
Glucocorticoids
Methotrexate
Tumor Necrosis Factor-alpha

Figure

• 

  Figure 1 (A) Livedo reticularis and subcutaneous nodules on the legs. (B) After the fourth injection of adalimumab, skin lesions on legs faded.

• 

  Figure 2 Histopathologic analysis of skin. (A) Intimal thickening with neutrophils and lymphocytes infiltration of medium-sized vessel in the fatty layer (H&E, ×100). (B) Thrombotic obliteration of vascular lumen (H&E, ×100).

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