关键词: Adult-onset Still's disease Dermatose neutrophilique Dermatose neutrophilique urticarienne Lupus erythematosus Lupus érythémateux Maladie de Still de l’adulte Neutrophilic dermatosis Neutrophilic urticarial dermatosis Schnitzler syndrome Syndrome de Schnitzler Urticaire Urticaria

Mesh : 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 Neutrophils / pathology Schnitzler Syndrome / complications Still's Disease, Adult-Onset / complications Sweet Syndrome / diagnosis Urticaria / diagnosis drug therapy etiology pathology Vasculitis / diagnosis

来  源:   DOI:10.1016/j.annder.2018.06.010   PDF(Sci-hub)

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.
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