ABSTRACT
Neutrophilic urticarial dermatosis (NUD) is a rare form of dermatosis. In clinical terms, it consists of a chronic or recurrent eruption comprising slightly elevated, pink to reddish plaques or macules. The elementary lesion lasts 24 to 48hours and resolves without leaving any residual pigmentation. Extra-cutaneous signs are common, particularly fever or arthralgia. At histopathology, the dermis contains dense neutrophilic interstitial infiltrate with leukocytoclasis, but without fibrinoid necrosis of vessel walls. NUD often occurs in a setting of underlying systemic disease. The most commonly associated diseases are adult-onset Still's disease, Schnitzler syndrome, lupus erythematosus and cryopyrin-associated periodic syndromes. Treatment of NUD depends on the clinical context. Dapsone and colchicine are often effective.
Subject(s)
Neutrophils/pathology , Urticaria/pathology , Arthralgia/etiology , Colchicine/therapeutic use , Cryopyrin-Associated Periodic Syndromes/complications , Dapsone/therapeutic use , Dermis/pathology , Diagnosis, Differential , Drug Eruptions/pathology , Drug Resistance , Fever/etiology , Histamine Antagonists/therapeutic use , Humans , Immunosuppressive Agents/therapeutic use , Lupus Erythematosus, Systemic/complications , Schnitzler Syndrome/complications , Still's Disease, Adult-Onset/complications , Sweet Syndrome/diagnosis , Urticaria/diagnosis , Urticaria/drug therapy , Urticaria/etiology , Vasculitis/diagnosisABSTRACT
Systemic urticaria are defined as urticaria, most often chronic, associated with systemic diseases. At present time, urticarial vasculitis and neutrophilic urticarial dermatosis associated to autoinflammatory syndromes are not considered to be subtypes of chronic spontaneous urticaria due to their distinctly clinical and histological characteristics as well different pathomechanisms. Sometimes, chronic urticaria is associated to thyroid autoimmunity. However, the majority of cases of chronic spontaneous urticaria have no discernible cause and further investigations are not necessary, as already suggested by some authors and French consensus conference more than 10 years ago.