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      Annular Leukocytoclastic Vasculitis: A New Feature of IgA Vasculitis

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          Abstract

          Background

          Annular erythema is a rare manifestation of leukocytoclastic vasculitis. It may be associated with various drugs, infections, malignancies, or systemic diseases.

          Case description

          A 36-year-old woman with no personal medical history presented with annular erythema with target lesions and petechial purpura. The patient had fever and joint arthralgia. A skin biopsy showed leukocytoclastic vasculitis with IgA deposits on direct immunofluorescence. The diagnosis of immunoglobulin A vasculitis with annular leukocytoclastic vasculitis was made. The patient showed global improvement with topical steroids without relapse.

          Conclusion

          An annular variant of leukocytoclastic vasculitis is a rare manifestation of immunoglobulin A vasculitis.

          LEARNING POINTS
          • Annular erythema may reveal an IgA vasculitis.

          • Annular leukocytoclastic vasculitis is a rare manifestation of IgA vasculitis.

          • This presentation is treated with topical steroids.

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          Most cited references15

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          Annular Lesions: Diagnosis and Treatment.

          Annular lesions can present in a variety of diseases. Knowledge of the physical appearance and history of presentation of these skin findings can help in the diagnosis. A pruritic, annular, erythematous patch that grows centrifugally should prompt evaluation for tinea corporis. Tinea corporis may be diagnosed through potassium hydroxide examination of scrapings. Recognizing erythema migrans is important in making the diagnosis of Lyme disease so that antibiotics can be initiated promptly. Plaque psoriasis generally presents with sharply demarcated, erythematous silver plaques. Erythema multiforme, which is due to a hypersensitivity reaction, presents with annular, raised lesions with central clearing. Lichen planus characteristically appears as planar, purple, polygonal, pruritic papules and plaques. Nummular eczema presents as a rash composed of coin-shaped papulovesicular erythematous lesions. Treatment is aimed at reducing skin dryness. Pityriasis rosea presents with multiple erythematous lesions with raised, scaly borders, and is generally self-limited. Urticaria results from the release of histamines and appears as well-circumscribed, erythematous lesions with raised borders and blanched centers. Annular lesions occur less commonly in persons with fixed drug eruptions, leprosy, immunoglobulin A vasculitis, secondary syphilis, sarcoidosis, subacute cutaneous lupus erythematosus, and granuloma annulare.
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            Recurrent annular erythema with purpura: a new variant of leucocytoclastic vasculitis responsive to dapsone.

            Annular lesions are rarely reported in the clinical spectrum of leucocytoclastic vasculitis, except in the acute haemorrhagic oedema of the skin. We report three patients who suffered from an extraordinary recurrent annular dermatitis, for 4 years in one case and for decades in the other two. The eruption was characterized by purpuric lesions that had a centrifugal evolution, creating target- or polycyclic patches disseminated on the limbs and trunk. The patients' general condition remained excellent during the attacks. All lesions spontaneously disappeared within 2 weeks, but recurred monthly. In all three cases, the histological changes were consistent with leucocytoclastic vasculitis. One patient had ulcerative colitis and another had a benign immunoglobulin A (IgA) monoclonal gammopathy. These two patients showed a good response to dapsone therapy. This dermatosis probably represents a new and rare variant of leucocytoclastic vasculitis.
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              Annular leukocytoclastic vasculitis associated with monoclonal gammopathy of unknown significance.

              Leukocytoclastic vasculitis is a condition characterized by necrotizing neutrophilic inflammation of small dermal blood vessels usually resulting in palpable purpuric lesions. Leukocytoclastic vasculitis may be secondary to a variety of medications and underlying disease processes, including infections, connective tissue disorders, and malignancies. We describe a patient with a monoclonal gammopathy of unknown significance in whom leukocytoclastic vasculitis developed, manifested by a few prominent annular plaques on the lower extremity. The rare association between monoclonal gammopathy of unknown significance and leukocytoclastic vasculitis as well as the unusual annular presentation of the disease in this patient is discussed, and the relevant literature is reviewed.
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                Author and article information

                Journal
                Eur J Case Rep Intern Med
                European Journal of Case Reports in Internal Medicine
                SMC Media Srl
                2284-2594
                2024
                03 April 2024
                : 11
                : 5
                : 004278
                Affiliations
                [1 ]Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Sud-Ile de France, Melun, France
                [2 ]Infectious Disease Department, Nord Franche-Comté Hospital, Trevenans, France
                [3 ]Department of Cardiology, Groupe Hospitalier Sud-Ile de France, Melun, France
                [4 ]Department of Internal Medicine and Reference Centre of Rare Disease, Assistance Publique des Hôpitaux de Paris (AP-HP), Cochin Hospital, Paris, France
                Author notes
                Corresponding author: Nabil Belfeki, e-mail: nabil.belfeki@ 123456ghsif.fr
                Article
                4278
                10.12890/2024_004278
                11073609
                38715881
                af9091ba-5349-4c73-9e9a-31539349fd8f
                © EFIM 2024

                This article is licensed under a Commons Attribution Non-Commercial 4.0 License

                History
                : 17 January 2024
                : 25 January 2024
                Categories
                Article

                annular erythema,ig-a mediated vasculitis,leukocytoclastic vasculitis,vasculitis,topical corticosteroids

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