Is chronic urticaria or urticarial vasculitis a diagnostic dilemma?
DOI:
https://doi.org/10.15574/SP.2024.138.133Keywords:
urticaria, vasculitis, autoantibodies, childrenAbstract
Introduction. Urticarial vasculitis (UV) is a rare disease that has two components: clinical manifestations of urticaria and histopathological signs of cutaneous leukocytoclastic vasculitis of small vessels, predominantly involving postcapillary venules. This condition is characterized by chronic or recurrent episodes of urticaria, each element of which lasts more than 24 hours and is accompanied by a feeling of pain and burning.
The aim is to reveal the key points of pathogenetic mechanisms, differential diagnosis and therapeutic tactics of UV based on a clinical case.
Clinical case. A clinical case of a 17-year-old boy with normocomplementemic UV is described. The patient's main complaint was a long-lasting rash (more than three weeks) with itching. From the anamnesis it is known that the provoking factors for the onset of the disease were an insect bite and the start of taking a new drug, namely vitamin K (two days before the onset of the disease). Throughout this time, the child was examined by various specialists and received treatment. Alternative diagnoses: bacterial folliculitis, viral exanthem, unspecified urticaria. There was no positive effect from the received treatment. The diagnosis of UV was made in the sixth week of the disease using a punch biopsy. Regression of the skin syndrome was achieved using a combination of antihistamine and antileukotriene drugs.
Conclusions. Performing a punch biopsy, which is currently the gold standard for diagnosis, allows us to solve the diagnostic dilemma: “UV or chronic urticaria”. Timely diagnosis helps to avoid false diagnoses and, as a result, incorrect treatment of UV. The description of this clinical case is a contribution to the disclosure of this globally complex problem.
The research was carried out in accordance with the principles of the Declaration of Helsinki. The informed consent of the child and child's parents was obtained for conducting the research.
No conflict of interest was declared by the authors.
References
Alberti-Violetti S, Berti E, Marzano AV. (2018). Cutaneous and systemic vasculitides in dermatology: a histological perspective. G Ital Dermatol Venereol. 153(2): 185-193. https://doi.org/10.23736/S0392-0488.18.05886-8; PMid:29368866
Caproni M, Verdelli A. (2019). An update on the nomenclature for cutaneous vasculitis. Curr Opin Rheumatol. 31(1): 46-52. https://doi.org/10.1097/BOR.0000000000000563; PMid:30394939
Chen HJ, Bloch KJ. (2001). Hypocomplementemic urticarial vasculitis, jaccoud's arthropathy, valvular heart disease, and reversible tracheal stenosis: a surfeit of syndromes. J Rheumatol. 28(2): 383-386. PMID: 11246684.
Davis MD, Brewer JD (2004) Urticarial vasculitis and hypocomplementemic urticarial vasculitis syndrome. Immunol Allergy Clin North Am. 24(2):183-213, https://doi.org/10.1016/j.iac.2004.01.007; PMid:15120147
Giang J, Seelen MAJ, van Doorn MBA, Rissmann R, Prens EP, Damman J. (2018). Complement Activation in Inflammatory Skin Diseases. Front Immunol. 16; 9: 639. https://doi.org/10.3389/fimmu.2018.00639; PMid:29713318 PMCid:PMC5911619
Gu SL, Jorizzo JL. (2021). Urticarial vasculitis. Int J Womens Dermatol. 29; 7(3): 290-297. https://doi.org/10.1016/j.ijwd.2021.01.021; PMid:34222586 PMCid:PMC8243153
Johnson EF, Wetter DA, Lehman JS, Hand JL, Davis DM, Tollefson MM. (2017). Leukocytoclastic vasculitis in children: clinical characteristics, subtypes, causes and direct immunofluorescence findings of 56 biopsy-confirmed cases. J Eur Acad Dermatol Venereol. 31(3): 544-549. https://doi.org/10.1111/jdv.13952; PMid:27580419
Kolkhir P, Bonnekoh H, Kocatürk E, Hide M, Metz M, Sánchez-Borges M et al. (2020). Management of urticarial vasculitis: A worldwide physician perspective. World Allergy Organ J. 13(3): 100107. https://doi.org/10.1016/j.waojou.2020.100107; PMid:32180892 PMCid:PMC7063238
Kolkhir P, Grakhova M, Bonnekoh H, Krause K, Maurer M. (2019). Treatment of urticarial vasculitis: A systematic review. J Allergy Clin Immunol. 143(2): 458-466. https://doi.org/10.1016/j.jaci.2018.09.007; PMid:30268388
Lee JS, Loh TH, Seow SC, Tan SH. (2007). Prolonged urticaria with purpura: the spectrum of clinical and histopathologic features in a prospective series of 22 patients exhibiting the clinical features of urticarial vasculitis. J Am Acad Dermatol. 56(6): 994-1005. https://doi.org/10.1016/j.jaad.2006.10.962; PMid:17504716
Marzano AV, Maronese CA, Genovese G, Ferrucci S, Moltrasio C, Asero R et al. (2022). Urticarial vasculitis: Clinical and laboratory findings with a particular emphasis on differential diagnosis. J Allergy Clin Immunol. 149(4): 1137-1149. https://doi.org/10.1016/j.jaci.2022.02.007; PMid:35396080
Morita TCAB, Criado PR, Criado RFJ, Trés GFS, Sotto MN. (2020). Update on vasculitis: overview and relevant dermatological aspects for the clinical and histopathological diagnosis - Part II. An Bras Dermatol. 95(4): 493-507. https://doi.org/10.1016/j.abd.2020.04.004; PMid:32527591 PMCid:PMC7335877
Okhotnikova OM, Oshlyanska OA. (2021). Uricar vasculitis in children. Asthma and allergy. 3: 16-28. https://doi.org/10.31655/2307-3373-2021-3-16-29
Sjöwall C, Skattum L, Olsson M, Nilsson A, Mohammad AJ. (2019). HUVS - rare but not harmless vasculitis associated with urticaria - Swedish study shows an annual incidence of 0.7 cases per million inhabitants [Hypocomplementemic urticarial vasculitis syndrome: a rare but not always benign condition]. The Lakar newspaper. 116: FR49.
Soylu A, Kavukçu S, Uzuner N, Olgaç N, Karaman O, Ozer E. (2001). Systemic lupus erythematosus presenting with normocomplementemic urticarial vasculitis in a 4-year-old girl. Pediatrician Int. 43(4): 420-422. https://doi.org/10.1046/j.1442-200X.2001.01404.x; PMid:11472591
Vallianou K, Skalioti C, Liapis G, Boletis JN, Marinaki S. (2020). A case report of hypocomplementemic urticarial vasculitis presenting with membranoproliferative glomerulonephritis. BMC Nephrol. 21(1): 351. https://doi.org/10.1186/s12882-020-02001-6; PMid:32811472 PMCid:PMC7433181
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