Neutrophilic urticarial dermatosis without systemic disease: case report

Abstract

Neutrophilic urticarial dermatosis (NUD) is an uncommon and not well understood disease. We report a 24-year-old female with persistent present with pruritic and painful urticarial plaques unresponsive to convential treatment. Histopathologically, it demonstrates a perivascular and interstitial neutrophilic infiltrate with leukocytoclasia without evidence of vasculitis or dermal edema consistent with neutrophilic urticarial dermatosis. Further investigations to rule out underlying autoimmune, autoinflammatory and gentic systemic disease were negative. Furthermore, the cutaneous eruption was resistant to multiple therapeutic interventions including colchicine and dapsone treatment, and show significant response to treatment with anakinra, interleukin-1 receptor antagonist. Based on literature review, the case reported here is the second case with clinical and pathologic features of NUD without systemic disease and the first case of NUD that showed resistant to colchicine and dapsone treatment, with adequate response to anakinra.