Review
Neutrophilic urticarial dermatosis: A reviewDermatose neutrophilique urticarienne

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Summary

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 48 hours 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.

Résumé

La dermatose neutrophilique urticarienne (DUN) est une dermatose rare. Cliniquement, il s’agit d’une éruption chronique ou récurrente, faite de macules ou de plaques discrètement surélevées, roses à rouges. La lésion élémentaire dure 24 à 48 heures et disparaît sans laisser de pigmentation résiduelle. Des signes extra-cutanés sont fréquents, en particulier de la fièvre ou des arthralgies. Histologiquement, le derme est le siège d’un infiltrat de polynucléaires neutrophiles denses, interstitiel avec de la leucocytoclasie mais sans nécrose fibrinoïde des parois vasculaires. La DUN survient fréquemment dans un contexte de maladie systémique sous-jacente. Les maladies les plus fréquemment associées sont la maladie de Still de l’adulte, le syndrome de Schnitzler, le lupus érythémateux et les syndromes périodiques associés à la cryopyrine. Le traitement de la DUN dépend du contexte clinique. La dapsone et la colchicine sont souvent efficaces.

Section snippets

Clinical description

The elementary lesion consists of a macule, papule or slightly raised plaque, seen primarily on the trunk, but also occasionally on the limbs (Fig. 1) and face. There is no impairment of the skin surface. Angioedema and involvement of the palms and soles occur more rarely. These lesions are less oedematous and less pruritic than those seen in classic urticaria, but their duration is similar (Table 1). In rare cases, Köbner phenomenon may occur, as well as an annular configuration or a halo of

Histological description

Diagnosis of NUD is based on a fairly dense neutrophilic dermal infiltrate of interstitial and perivascular localisation (Fig. 2a), comprising leukocytoclasis but without vasculitis, in other words fibrinoid necrosis of the vessel walls found in autoimmune vasculitis and urticarial vasculitis. The typical appearance is that of neutrophils in Indian file next to collagen bands (Fig. 2b). Epithelial tropism can also occur, particularly in sweat glands [3]. The absence of oedema in the superficial

Associated signs

In NUD, laboratory signs of inflammatory syndrome may be seen, with elevated CRP, neutrophilic hyperleukocytosis or increased erythrocyte sedimentation rate (ESR). The intensity of this inflammatory syndrome varies according to clinical setting.

No signs of arthritis are discernible in X-rays of painful joints, if performed, or using other bone-joint investigation techniques. As in cases of abdominal or thoracic pain, the various laboratory tests are generally inconclusive.

Associated diseases

Diagnosis of NUD requires screening for systemic disease (Box 1), since this association is common though not inevitable (NUD is a separate entity). Screening should be performed at the initial diagnosis and during the course of the disease if any worsening is seen. NUD may also occur during an existing systemic disease. Commonly associated diseases are adult-onset Still's disease, Schnitzler syndrome, lupus erythematosus and CAPS (cryopyrin-associated periodic syndrome) [1], [4], [5].

Chronic spontaneous urticaria

Chronic spontaneous urticaria (CSU) involves superficial or deep urticaria ongoing for more than 10 weeks, with no evidence of inflammatory disease, vasculitis or bradykinin angioedema, and with negative induced-urticaria challenge tests [14]. It may be superficial, consisting of pink to erythematous macules, papules and barely raised plaques that are pruritic, fleeting and migratory. It may also be deep and characterised by firm, pale and poorly delimited swelling, with little or no pruritus.

Management

Treatment of NUD depends on the clinical context in which it occurs. Classically, antihistamines are ineffective, but they should be tried at least initially.

Where NUD occurs concomitantly with lupus erythematosus, immunosuppressants are ineffective and produce many adverse effects [4]. Neutrophil migration inhibitors must be initiated, particularly colchicine or dapsone. Dapsone must be administered at the effective dosage, generally between 50 and 200 mg/d depending on patients. Regular

Disclosure of interest

The authors declare that they have no competing interest.

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