Neutrophilic urticarial dermatosis: a novel association with poststreptococcal rheumatic disease

Abstract

Neutrophilic urticarial dermatosis (NUD), a particular clinical and histological entity, can provide a strong pointer to underlying systemic disease, most frequently rheumatological diseases. We report the first case of NUD in association with a post-streptococcal rheumatic disease, with symptoms including recurrent sore throat, raised antistreptolysin O titre, persistent transient urticaria, polyarthralgia, rheumatic mitral valve disease and Jaccoud arthropathy. Histologically, NUD is characterized by an intense superficial and deep neutrophilic interstitial and perivascular infiltrate, without significant oedema or blood vessel damage. These neutrophils may have a tendency to concentrate along the basement membrane and extend into the epidermis, hair follicles, sebaceous glands and sweat glands (a feature termed 'neutrophilic epitheliotropism'). Clinicians should remain cognizant of NUD, and in particular its frequent association with an underlying inflammatory disorder.

Neutrophilic urticarial dermatosis: an entity bridging monogenic and polygenic autoinflammatory disorders, and beyond

“…Amongst those, two cases of serum sickness‐like drug reaction (SSLR) have been reported . It has also been reported as a feature of poststreptococcal rheumatological disease . Furthermore, associations with primary Sjögren syndrome, primary biliary cirrhosis or inflammatory bowel disease are also described .…”

“…Lupus erythematosus is a polygenic autoimmune disease characterized by the presence of non-organ-specific humoral Serum sickness-like drug reaction 2 Nguyen et al 13 Poststreptococcal rheumatic disease 1 Salam et al 18 Acral acquired cutis laxa associated with IgA multiple myeloma autoantibodies. Recently, the role of neutrophils in the pathophysiology of LE has emerged.…”

“…13 It has also been reported as a feature of poststreptococcal rheumatological disease. 18 Furthermore, associations with primary Sj€ ogren syndrome, primary biliary cirrhosis or inflammatory bowel disease are also described. 11,16 A few cases of NUD in the context of IgA gammopathy, the isotype most commonly associated with neutrophilic dermatoses 31,32 have also been reported.…”

“…Since its original description, NUD has been accepted as an autonomous entity. Numerous new cases have been reported, and associated diseases have been confirmed and expanded . Table summarizes the associated diseases that have been published since our original publication in 2009.…”

“…Numerous new cases have been reported, and associated diseases have been confirmed and expanded. 1,[3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Table 1 summarizes the associated diseases that have been published since our original publication in 2009. NUD can also occur without any underlying disease, as an entity on its own.…”