Neutrophilic Urticarial Dermatosis With Angioedema: A Diagnostic and Therapeutic Challenge

Neutrophilic urticarial dermatosis (NUD) is a rare subtype of neutrophilic dermatoses (NDs), presenting with recurrent erythematous papules and plaques often resistant to conventional therapies and associated with systemic symptoms such as fever and polyarthritis. Here, we report the case of a 32-year-old female with chronic pruritus, hives, and angioedema, unresponsive to antihistamines and progressing to diffuse urticarial lesions. Initial differential diagnoses included chronic urticaria, systemic lupus erythematosus, and Schnitzler syndrome. Laboratory testing showed elevated C-reactive protein and weakly positive antinuclear antibodies (ANA), while histopathology confirmed NUD with neutrophilic infiltrates and absence of vasculitis. Management involved methylprednisolone and cetirizine, with the eventual introduction of colchicine, leading to temporary symptomatic resolution. However, with eventual tapering of methylprednisolone, symptoms began to recur, prompting the use of immunosuppressive therapy. This case underscores the importance of histopathological analysis in diagnosing NUD along with refractory disease management. Further research on the pathogenesis of NUD, including genetic testing and screening for underlying autoinflammatory syndromes, may refine treatment protocols and enhance patient outcomes.