ReviewNeutrophilic urticarial dermatosis: A reviewDermatose neutrophilique urticarienne
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.
References (14)
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Annular urticarial lesions
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2022, Journal of Allergy and Clinical ImmunologyCitation Excerpt :As a manifestation of SLE, NUD tends to present with fever and joint pain, leading to its misinterpretation as a lupus flare. Antinuclear antibodies are always positive in LE-associated NUD, particularly anti-Ro/SSA and anti-La/SSB,90 as opposed to LE-associated HUV in which anti-C1q autoantibodies play a major role.91 Differently from SLE- or rheumatoid arthritis–associated vasculitis, HUV or NUV is mostly confined to postcapillary venules.