Neutrophilic urticarial dermatosis (NUD) in probable adult-onset Still disease responding to anakinra.

J Clin Rheumatol

From the Departments of *Rheumatology and †Dermatology, Center of Inflammation Medicine, University Medical Center Schleswig-Holstein, Campus Kiel, Germany. The authors declare no conflict of interest.

Published: March 2014

Differential diagnosis of urticarial skin lesions not representing classic urticaria remains a challenge. Long-lasting nonpruritic urticarial plaques and the histopathologic finding of a dense infiltrate with neutrophil granulocytes are indicative for a rare entity within a group of diseases termed neutrophilic dermatoses. Here, we report a case of neutrophilic urticarial dermatosis characterized by nonpruritic urticarial skin lesions, histopathologic changes resembling Sweet syndrome, and simultaneous Still syndrome. After treatment with conventional immunosuppressives including systemic corticosteroids without disease control, the patient responded to anakinra therapy within days achieving complete remission of skin lesions and systemic symptoms including fever and high C-reactive protein.

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http://dx.doi.org/10.1097/RHU.0000000000000078DOI Listing

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