{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T10:24:37Z","timestamp":1787048677851,"version":"build-2736575974"},"reference-count":54,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T00:00:00Z","timestamp":1787011200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dermatopathology"],"abstract":"<jats:p>Vasculitis encompasses a heterogeneous group of diseases characterized by the inflammation of blood vessels. In children, the diagnosis of vasculitis with cutaneous involvement relies on a combination of clinical evaluation, histopathological examination, and, in some cases, genetic investigations. Diagnosing pediatric vasculitis remains particularly challenging due to overlapping clinical manifestations, variable disease courses, and the evolving nature of histological features. Histopathological assessment aims to confirm inflammation of the vessel walls\u2014either readily visible at low magnification or requiring serial sections\u2014and to characterize the inflammatory infiltrate and other key features that aid in classifying the vasculitis subtype. Accurate diagnosis requires ongoing dialog and collaboration among multiple specialists. In this article, we present the clinical and histopathological features of the most common pediatric vasculitides\u2014including IgA vasculitis and polyarteritis nodosa\u2014as well as newly recognized entities such as COVID-19-associated vasculitis and monogenic vasculitides.<\/jats:p>","DOI":"10.3390\/dermatopathology13030037","type":"journal-article","created":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T09:06:41Z","timestamp":1787044001000},"page":"37","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Cutaneous Pediatric Vasculitis: A Clinico-Histopathological Overview of Common and Novel Entities"],"prefix":"10.3390","volume":"13","author":[{"given":"Anne","family":"Welfringer-Morin","sequence":"first","affiliation":[{"name":"Department of Dermatology, H\u00f4pital Necker-Enfants Malades, Assistance Publique\u2014H\u00f4pitaux de Paris, 75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2468-3910","authenticated-orcid":false,"given":"St\u00e9phanie","family":"Leclerc-Mercier","sequence":"additional","affiliation":[{"name":"Department of Pathology, H\u00f4pital Necker-Enfants Malades, Assistance Publique\u2014H\u00f4pitaux de Paris, 75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,8,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1002\/art.1780370206","article-title":"Nomenclature of Systemic Vasculitides","volume":"37","author":"Jennette","year":"1994","journal-title":"Arthritis Rheum."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1002\/art.37715","article-title":"2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides","volume":"65","author":"Jennette","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"798","DOI":"10.1136\/ard.2009.116657","article-title":"EULAR\/PRINTO\/PRES Criteria for Henoch-Sch\u00f6nlein Purpura, Childhood Polyarteritis Nodosa, Childhood Wegener Granulomatosis and Childhood Takayasu Arteritis: Ankara 2008. Part II: Final Classification Criteria","volume":"69","author":"Ozen","year":"2010","journal-title":"Ann. Rheum. Dis."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1197","DOI":"10.1016\/S0140-6736(02)11279-7","article-title":"Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins","volume":"360","author":"Dolezalova","year":"2002","journal-title":"Lancet"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1007\/s12016-017-8626-3","article-title":"Cutaneous Manifestations of Small-Vessel Leukocytoclastic Vasculitides in Childhood","volume":"53","author":"Lava","year":"2017","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1007\/s10067-020-05166-5","article-title":"Multisystemic Manifestations of IgA Vasculitis","volume":"40","author":"Du","year":"2021","journal-title":"Clin. Rheumatol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1607","DOI":"10.1093\/rheumatology\/kez041","article-title":"European Consensus-Based Recommendations for Diagnosis and Treatment of Immunoglobulin A Vasculitis-the SHARE Initiative","volume":"58","author":"Ozen","year":"2019","journal-title":"Rheumatology"},{"key":"ref_8","unstructured":"Patterson, J.W. (2024). Weedon\u2019s Skin Pathology. 9. The Vasculopathic Reaction Pattern, Elsevier. [6th ed.]."},{"key":"ref_9","unstructured":"Calonje, J.E., Brenn, T., Lazar, A.J., and Billings, S.D. (2019). McKee\u2019s Pathology of the Skin, Elsevier\u2014OHCE. [5th ed.]. Elsevier eBooks+."},{"key":"ref_10","first-page":"48","article-title":"Immunofluorescence directe en dermatologie: Principales indications","volume":"508","author":"Menzinger","year":"2019","journal-title":"Rev. Francoph. Lab."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1135","DOI":"10.1002\/art.1780330812","article-title":"The American College of Rheumatology 1990 Criteria for the Classification of Vasculitis. Summary","volume":"33","author":"Fries","year":"1990","journal-title":"Arthritis Rheum."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1186\/s13075-016-0959-4","article-title":"IgA Vasculitis in Adults: The Performance of the EULAR\/PRINTO\/PRES Classification Criteria in Adults","volume":"18","author":"Rotar","year":"2016","journal-title":"Arthritis Res. Ther."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1159\/000519009","article-title":"Skin Eruptions in Acute Hemorrhagic Edema of Young Children: Systematic Review of the Literature","volume":"238","author":"Bronz","year":"2022","journal-title":"Dermatology"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"4133","DOI":"10.1007\/s00431-023-05098-7","article-title":"Accurate Diagnosis of Acute Hemorrhagic Edema of Infancy: A French Multicenter Observational Study","volume":"182","author":"Leducq","year":"2023","journal-title":"Eur. J. Pediatr."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"684","DOI":"10.1016\/j.jaad.2008.06.005","article-title":"Acute Hemorrhagic Edema of Young Children (Cockade Purpura and Edema): A Case Series and Systematic Review","volume":"59","author":"Fiore","year":"2008","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1016\/j.jpeds.2014.09.048","article-title":"Acute Hemorrhagic Edema of Infancy: A Rare Cause of Purpuric Exanthema","volume":"166","author":"Oliveira","year":"2015","journal-title":"J. Pediatr."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1250","DOI":"10.1111\/ddg.14271","article-title":"Manifestations, clinical course and prognostic markers in cutaneous polyarteritis nodosa","volume":"18","author":"Bielsa","year":"2020","journal-title":"J. Dtsch. Dermatol. Ges."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1016\/j.jpeds.2004.06.046","article-title":"Juvenile polyarteritis: Results of a multicenter survey of 110 children","volume":"145","author":"Ozen","year":"2004","journal-title":"J. Pediatr."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1002\/acr.24633","article-title":"2021 American College of Rheumatology\/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa","volume":"73","author":"Chung","year":"2021","journal-title":"Arthritis Care Res."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1097\/MD.0b013e3182115508","article-title":"Correlation of Livedo Racemosa, Cutaneous Inflammatory Plaques, and Antiphospholipid Antibodies in Patients with Cutaneous Polyarteritis Nodosa","volume":"90","author":"Kawakami","year":"2011","journal-title":"Medicine"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"e927","DOI":"10.1161\/CIR.0000000000000484","article-title":"Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals from the American Heart Association","volume":"135","author":"McCrindle","year":"2017","journal-title":"Circulation"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"672","DOI":"10.1093\/rheumatology\/key344","article-title":"European Consensus-Based Recommendations for the Diagnosis and Treatment of Kawasaki Disease\u2014The SHARE Initiative","volume":"58","author":"Groot","year":"2019","journal-title":"Rheumatology"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1016\/j.revmed.2023.06.002","article-title":"French National Diagnostic and Care Protocol for Kawasaki Disease","volume":"44","author":"Galeotti","year":"2023","journal-title":"Rev. Med. Interne"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1034\/j.1600-0560.2003.00033.x","article-title":"Cutaneous vasculitis: A review","volume":"30","author":"Crowson","year":"2003","journal-title":"J. Cutan. Pathol."},{"key":"ref_25","first-page":"476","article-title":"Immunopathology of skin lesions in Kawasaki disease","volume":"40","author":"Sugawara","year":"1991","journal-title":"Arerugi"},{"key":"ref_26","doi-asserted-by":"crossref","unstructured":"Maronese, C.A., Zelin, E., Avallone, G., Moltrasio, C., Romagnuolo, M., Ribero, S., Quaglino, P., and Marzano, A.V. (2022). Cutaneous Vasculitis and Vasculopathy in the Era of COVID-19 Pandemic. Front. Med., 9.","DOI":"10.3389\/fmed.2022.996288"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1111\/ced.14483","article-title":"ESPD Group for the Skin Manifestations of COVID-19. Skin Manifestations of COVID-19 in Children: Part 3","volume":"46","author":"Andina","year":"2021","journal-title":"Clin. Exp. Dermatol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1111\/cup.14099","article-title":"A Clinicopathological Description of COVID-19-Induced Chilblains (COVID-Toes) Correlated with a Published Literature Review","volume":"49","author":"Kolivras","year":"2022","journal-title":"J. Cutan. Pathol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1056\/NEJMoa1307362","article-title":"Mutant Adenosine Deaminase 2 in a Polyarteritis Nodosa Vasculopathy","volume":"370","author":"Elkan","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1056\/NEJMoa1307361","article-title":"Early-Onset Stroke and Vasculopathy Associated with Mutations in ADA2","volume":"370","author":"Zhou","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_31","doi-asserted-by":"crossref","unstructured":"Barron, K.S., Aksentijevich, I., Deuitch, N.T., Stone, D.L., Hoffmann, P., Videgar-Laird, R., Soldatos, A., Bergerson, J., Toro, C., and Cudrici, C. (2022). The Spectrum of the Deficiency of Adenosine Deaminase 2: An Observational Analysis of a 60 Patient Cohort. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.811473"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1186\/s13023-023-02721-6","article-title":"A Wide Spectrum of Phenotype of Deficiency of Deaminase 2 (DADA2): A Systematic Literature Review","volume":"18","author":"Maccora","year":"2023","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1230","DOI":"10.1001\/jamadermatol.2015.1635","article-title":"Dermatologic Features of ADA2 Deficiency in Cutaneous Polyarteritis Nodosa","volume":"151","author":"Zavialov","year":"2015","journal-title":"JAMA Dermatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"202","DOI":"10.3390\/dermatopathology8020026","article-title":"Histologic Patterns and Clues to Autoinflammatory Diseases in Children: What a Cutaneous Biopsy Can Tell Us","volume":"8","author":"Kolivras","year":"2021","journal-title":"Dermatopathology"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1056\/NEJMoa1312625","article-title":"Activated STING in a Vascular and Pulmonary Syndrome","volume":"371","author":"Liu","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"5516","DOI":"10.1172\/JCI79100","article-title":"Inherited STING-activating mutation underlies a familial inflammatory syndrome with lupus-like manifestations","volume":"124","author":"Jeremiah","year":"2014","journal-title":"J. Clin. Investig."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1016\/j.jaip.2020.11.007","article-title":"Overview of STING-Associated Vasculopathy with Onset in Infancy (SAVI) Among 21 Patients","volume":"9","author":"Hadchouel","year":"2021","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"577918","DOI":"10.3389\/fped.2020.577918","article-title":"Stimulator of interferon genes-associated vasculopathy with onset in infancy: A systematic review of case reports","volume":"8","author":"Dai","year":"2020","journal-title":"Front. Pediatr."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1002\/art.42087","article-title":"The 2021 European Alliance of Associations for Rheumatology\/American College of Rheumatology Points to Consider for Diagnosis and Management of Autoinflammatory Type I Interferonopathies: CANDLE\/PRAAS, SAVI, and AGS","volume":"74","author":"Lamot","year":"2022","journal-title":"Arthritis Rheumatol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1007\/s10875-019-00645-0","article-title":"Efficacy and Adverse Events During Janus Kinase Inhibitor Treatment of SAVI Syndrome","volume":"39","author":"Volpi","year":"2019","journal-title":"J. Clin. Immunol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1016\/j.autrev.2017.11.014","article-title":"Childhood- versus adult-onset ANCA-associated vasculitides: A nested, matched case-control study from the French Vasculitis Study Group Registry","volume":"17","author":"Ludici","year":"2018","journal-title":"Autoimmun. Rev."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1097\/00000478-199502000-00005","article-title":"Cutaneous pathology in Wegener\u2019s granulomatosis. A clinicopathologic study of 75 biopsies in 46 patients","volume":"19","author":"Barksdale","year":"1995","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1007\/s12016-020-08788-4","article-title":"Cutaneous Vasculitis: Review on Diagnosis and Clinicopathologic Correlations","volume":"61","author":"Frumholtz","year":"2021","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1016\/S0190-9622(97)80125-9","article-title":"Cutaneous manifestations of Churg-Strauss syndrome: A clinicopathologic correlation","volume":"37","author":"Davis","year":"1997","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"144","DOI":"10.2340\/00015555-0034","article-title":"Cutaneous manifestations in patients with microscopic polyangiitis: Two case reports and a minireview","volume":"86","author":"Kawakami","year":"2006","journal-title":"Acta Derm.-Venereol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1136\/annrheumdis-2015-208491","article-title":"Consensus classification criteria for paediatric Beh\u00e7et\u2019s disease from a prospective observational cohort: PEDBD","volume":"75","author":"Shahram","year":"2016","journal-title":"Ann. Rheum. Dis."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1016\/S0190-9622(97)80318-0","article-title":"Cutaneous vasculitis in Beh\u00e7et\u2019s disease: A clinical and histopathologic study of 20 patients","volume":"36","author":"Chen","year":"1997","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1097\/00000372-200010000-00001","article-title":"Histopathologic Features of Erythema Nodosum\u2013Like Lesions in Beh\u00e7et Disease: A Comparison with Erythema Nodosum Focusing on the Role of Vasculitis","volume":"22","author":"Kim","year":"2000","journal-title":"Am. J. Dermatopathol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"929","DOI":"10.1046\/j.1365-4362.1998.00474.x","article-title":"The histopathology of pathergy: A chronologic study of skin hyperreactivity in Beh\u00e7et\u2019s disease","volume":"37","author":"Ergun","year":"1998","journal-title":"Int. J. Dermatol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"872313","DOI":"10.3389\/fped.2022.872313","article-title":"An Update on Childhood-Onset Takayasu Arteritis","volume":"10","author":"Aeschlimann","year":"2022","journal-title":"Front. Pediatr."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"2246","DOI":"10.1093\/rheumatology\/keaa569","article-title":"Clinical course of 602 patients with Takayasu\u2019s arteritis: Comparison between Childhood-onset versus adult onset disease","volume":"60","author":"Danda","year":"2021","journal-title":"Rheumatology"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1016\/j.semarthrit.2020.10.013","article-title":"Childhood-onset versus adult-onset Takayasu arteritis: A study of 141 patients from Turkey","volume":"51","author":"Karabacak","year":"2021","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"998","DOI":"10.1016\/S0190-9622(87)70289-8","article-title":"Cutaneous manifestations of Takayasu\u2019s arteritis","volume":"17","author":"Perniciaro","year":"1987","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_54","first-page":"266","article-title":"Cutaneous manifestations of Takayasu arteritis. A retrospective study of 80 cases","volume":"181","author":"Frances","year":"1990","journal-title":"Dermatology"}],"container-title":["Dermatopathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2296-3529\/13\/3\/37\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T09:29:59Z","timestamp":1787045399000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2296-3529\/13\/3\/37"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,18]]},"references-count":54,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2026,9]]}},"alternative-id":["dermatopathology13030037"],"URL":"https:\/\/doi.org\/10.3390\/dermatopathology13030037","relation":{},"ISSN":["2296-3529"],"issn-type":[{"value":"2296-3529","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,18]]}}}