Diagnostic value of skin biopsy in autoinflammatory diseases for patients with recurrent fever and urticarial eruption.

Abstract

Recurrent fever is defined by two episodes of fever separated by a free interval of at least two weeks.1 Diseases causing recurrent fever may be classified into three categories: infections, neoplasia, and non-infectious inflammations. Recurrent fever may be associated with autoinflammatory diseases like Schnitzler syndrome, NLRP3-associated autoinflammatory diseases (NLRP3-AID, formerly cryopyrin associated periodic syndrome or CAPS), or adult onset Still's disease (AOSD).2 Those diseases are characterized by an urticarial eruption with pale pink macules and slightly elevated papules and plaques lasting 24 to 48 hours.3 Histopathology typically shows a neutrophilic urticarial dermatosis (NUD), a dense perivascular and interstitial neutrophilic infiltrate with leukocytoclasia but without vasculitis or dermal oedema.4,5.