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Clinical and pathological evaluation of patients with leukocytoclastic vasculitis: a retrospective single-center rheumatology experience

Year 2025, Volume: 7 Issue: 4, 485 - 491, 28.07.2025
https://doi.org/10.38053/acmj.1731053

Abstract

Aims: This study aimed to evaluate the clinical and pathological characteristics of adult patients with leukocytoclastic vasculitis (LCV) and to assess the diagnostic impact of direct immunofluorescence (DIF) test in rheumatology practice.
Methods: The study included 102 adult patients admitted to the rheumatology clinic with palpable purpura between June 2020 and April 2025. All of the patients have a DIF test and a positive skin biopsy for LCV. The clinical and histopathological characteristics of the patients were retrospectively reviewed.
Results: After all diagnostic tests, 84 patients (82.4%) were classified as immunoglobulin A vasculitis (IgAV) and 11 (10.8%) as single-organ cutaneous small vessel vasculitis (SoCSVV). Sixty-five patients (63.7%) had comorbid diseases; endocrine-metabolic (36.3%) and cardiovascular diseases (30.4%) were the most common. Eighty-four patients (82.3%) received corticosteroids at various doses, and 41 (40.1%) received steroid-sparing agent. The DIF test supported the final clinical diagnosis in 55 patients (54%), was negative in 27 patients (26.4%), and revealed non-specific findings in 20 patients (19.6%). DIF results led to a change in the clinical pre-diagnosis in only 14 patients (13.7%). No significant differences were found between the two groups when comparing the laboratory and clinical findings at presentation in patients with skin-limited systemic vasculitis and those with SoCSVV.
Conclusion: Leukocytoclastic vasculitides that are assessed in adult rheumatology clinics are highly likely to be part of systemic vasculitides, particularly IgAV. Most adults with LCV have comorbidities and need corticosteroids. In clinical practice, the impact of the DIF test on classifying patients with LCV is limited.

References

  • Caproni M, Verdelli A. An update on the nomenclature for cutaneous vasculitis. Curr Opin Rheumatol. 2019;31(1):46-52. doi:10.1097/BOR. 0000000000000563
  • Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. doi:10.1002/art.37715
  • Loricera J, Blanco R, Ortiz-Sanjuán F, et al. Single-organ cutaneous small-vessel vasculitis according to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides: a study of 60 patients from a series of 766 cutaneous vasculitis cases. Rheumatology (Oxford). 2015;54(1):77-82. doi:10.1093/rheumatology/keu295
  • Bodakçi E. Clinical study on single-organ cutaneous small vessels vasculitis: a retrospective observational study. Eur Rev Med Pharmacol Sci. 2024;28(2):534-541. doi:10.26355/eurrev_202401_35051
  • Davarci E, Afşar FŞ, Özkavruk Eliyatkın N. Evaluation of clinicopathological characteristics in single-organ cutaneous small vessel vasculitis. Güncel Tıbbi Araştırmaları Dergisi. 2025;5(1):22-36.
  • Sunderkötter CH, Zelger B, Chen KR, et al. Nomenclature of cutaneous vasculitis: dermatologic addendum to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.Arthritis Rheumatol. 2018;70(2):171-184. doi:10.1002/art.40375
  • Jessop SJ. Cutaneous leucocytoclastic vasculitis: a clinical and aetiological study. Br J Rheumatol. 1995;34(10):942-945. doi:10.1093/rheumatology/34.10.942
  • Johnson EF, Wetter DA, Lehman JS, Hand JL, Davis DMR, Tollefson MM. Leukocytoclastic vasculitis in children: clinical characteristics, subtypes, causes and direct immunofluorescence findings of 56 biopsy-confirmed cases. J Eur Acad Dermatol Venereol. 2017;31(3):544-549. doi: 10.1111/jdv.13952
  • Piram M, Mahr A. Epidemiology of immunoglobulin A vasculitis (Henoch-Schönlein): current state of knowledge. Curr Opin Rheumatol. 2013;25(2):171-178. doi:10.1097/BOR.0b013e32835d8e2a
  • Gupta S, Handa S, Kanwar AJ, Radotra BD, Minz RW. Cutaneous vasculitides: clinico-pathological correlation. Indian J Dermatol Venereol Leprol. 2009;75(4):356–62
  • Ruperto N, Ozen S, Pistorio A, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: overall methodology and clinical characterisation. Ann Rheum Dis. 2010;69(5):790-797. doi:10.1136/ard.2009.116624
  • Sais G, Vidaller A, Jucglà A, Servitje O, Condom E, Peyri J. Prognostic factors in leukocytoclastic vasculitis: a clinicopathologic study of 160 patients. Arch Dermatol. 1998;134(3):309-315. doi:10.1001/archderm. 134.3.309
  • Bouiller K, Audia S, Devilliers H, et al. Etiologies and prognostic factors of leukocytoclastic vasculitis with skin involvement: a retrospective study in 112 patients. Medicine (Baltimore). 2016;95(28):e4238. doi:10. 1097/MD.0000000000004238
  • García-Porrúa C, González-Gay MA. Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch-Schönlein purpura in adults. Semin Arthritis Rheum. 1999;28(6):404-412. doi:10.1016/s0049-0172(99)80006-7
  • Pastuszczak M, Celińska-Löwenhoff M, Sułowicz J, Wojas-Pelc A, Musiał J. Clinical study on single-organ cutaneous small vessels vasculitis (SoCSVV). Medicine (Baltimore). 2017;96(12):e6376. doi:10. 1097/MD.0000000000006376
  • Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M. Cutaneous vasculitis in children and adults. Associated diseases and etiologic factors in 303 patients. Medicine (Baltimore). 1998;77(6):403-418. doi:10.1097/00005792-199811000-00007
  • Villatoro-Villar M, Crowson CS, Warrington KJ, Makol A, Ytterberg SR, Koster MJ. Clinical characteristics of biopsy-proven IgA vasculitis in children and adults: a retrospective cohort study. Mayo Clin Proc. 2019;94(9):1769-1780. doi:10.1016/j.mayocp.2019.04.034
  • Nossent J, Raymond W, Isobel Keen H, Preen D, Inderjeeth C. Morbidity and mortality in adult-onset IgA vasculitis: a long-term population-based cohort study. Rheumatology (Oxford). 2021;61(1):291-298. doi:10.1093/rheumatology/keab312
  • Boesen EI, Kakalij RM. Autoimmune-mediated renal disease and hypertension. Clin Sci (Lond). 2021;135(17):2165-2196. doi:10.1042/CS 20200955
  • 20. Huang X, Wu X, Le W, et al. Renal prognosis and related risk factors for Henoch-Schönlein purpura nephritis: a chinese adult patient cohort. Sci Rep. 2018;8(1):5585. doi:10.1038/s41598-018-23638-2
  • Kaynar Özçelik G, Içaçan OC, Yalçin Mutlu M, Yildirim F, Bes C. Palpable purpura: is it associated with vasculitis or not? A single-center experience. Clin Rheumatol. 2022;41(5):1493-1498. doi:10.1007/s10067-022-06075-5
  • Younger DS, Carlson A. Dermatologic aspects of systemic vasculitis.Neurol Clin. 2019;37(2):465-473. doi:10.1016/j.ncl.2019.01.017
  • Fraticelli P, Benfaremo D, Gabrielli A. Diagnosis and management of leukocytoclastic vasculitis. Intern Emerg Med. 2021;16(4):831-841. doi: 10.1007/s11739-021-02688-x
  • Arora A, Wetter DA, Gonzalez-Santiago TM, Davis MDP, Lohse CM. Incidence of leukocytoclastic vasculitis, 1996 to 2010: a population-based study in Olmsted County, Minnesota. Mayo Clin Proc. 2014;89(11): 1515-1524. doi:10.1016/j.mayocp.2014.04.015
  • Feasel P, Billings SD, Bergfeld WF, Piliang MP, Fernandez AP, Ko JS. Direct immunofluorescence testing in vasculitis-a single institution experience with Henoch-Schönlein purpura. J Cutan Pathol. 2018;45(1): 16-22. doi:10.1111/cup.13054
  • Nandeesh B, Tirumalae R. Direct immunofluorescence in cutaneous vasculitis: experience from a referral hospital in India. Indian J Dermatol. 2013;58(1):22-25. doi:10.4103/0019-5154.105280
  • Frumholtz L, Laurent-Roussel S, Lipsker D, Terrier B. Cutaneous vasculitis: review on diagnosis and clinicopathologic correlations. Clin Rev Allergy Immunol. 2021;61(2):181-193. doi:10.1007/s12016-020-08788-4
  • Lath K, Chatterjee D, Saikia UN, et al. Role of direct immunofluorescence in cutaneous small-vessel vasculitis: experience from a tertiary center. Am J Dermatopathol. 2018;40(9):661-666. doi:10.1097/DAD. 0000000000001170
  • Barnadas MA, Pérez E, Gich I, et al. Diagnostic, prognostic and pathogenic value of the direct immunofluorescence test in cutaneous leukocytoclastic vasculitis. Int J Dermatol. 2004;43(1):19-26. doi:10.1111/j.1365-4632.2004.01714.x
  • Alalwani M, Billings SD, Gota CE. Clinical significance of immunoglobulin deposition in leukocytoclastic vasculitis: a 5-year retrospective study of 88 patients at cleveland clinic. Am J Dermatopathol. 2014;36(9):723-729. doi:10.1097/DAD.0000000000000122
  • Lehman JS, Ferringer TC, Fung MA, Cassarino DS, Shalin SC. Diagnostic utility of direct immunofluorescence test panels for cutaneous vasculitis: a scoping review. J Cutan Pathol. 2024;51(12):987-999. doi:10.1111/cup.14722

Lökositoklastik vaskülitli hastaların klinik ve patolojik özelliklerinin değerlendirilmesi: tek merkez romatoloji deneyimi

Year 2025, Volume: 7 Issue: 4, 485 - 491, 28.07.2025
https://doi.org/10.38053/acmj.1731053

Abstract

Amaç: Bu çalışmada lökositoklastik vaskülitli (LCV) yetişkin hastaların klinik ve patolojik özelliklerinin incelenmesi ve romatoloji pratiğinde direkt immünofloresan (DIF) testin tanısal katkısının değerlendirilmesi amaçlandı.
Yöntem: Çalışmaya Haziran 2020 ile Nisan 2025 arasında palpabl purpura ile romatoloji kliniğine başvuran, deri biyopsisi ile LCV tanısı almış ve DIF testi yapılmış 102 yetişkin hasta dahil edildi. Hastaların klinik ve histopatolojik özellikleri retrospektif olarak incelendi.
Bulgular: Tüm tanı testlerinden sonra 84 hasta (%82,4) immünoglobulin A vasküliti (IgAV) ve 11 hasta (%10,8) tek organ kutanöz küçük damar vasküliti (SoCSVV) olarak sınıflandırılmıştı. Altmış beş hastada (%63,7) komorbid hastalık vardı; endokrin-metabolik (%36,3) ve kardiyovasküler hastalıklar (%30,4) en yaygın komorbiditelerdi. Seksen dört hasta (%82,3) çeşitli dozlarda kortikosteroid almıştı ve 41 hastaya (%40,1) steroid ayırıcı tedavi verilmişti. DIF testi 55 hastada (%54) nihai klinik tanıyı desteklerken, 20 hastada (%19,6) nonspesifik bulgular gösterdi ve 27 hastada (%26.4) negatif idi. DIF sonuçları yalnızca 14 hastada (%13,7) klinik ön tanıda değişikliğe yol açmıştı. Deriye sınırlı sistemik vaskülitli hastalar ile SoCSVV'li hastaların başvurudaki laboratuvar ve klinik bulguları karşılaştırıldığında iki grup arasında anlamlı bir fark saptanmadı.
Sonuç: Erişkin romatoloji kliniklerinde değerlendirilen lökositoklastik vaskülitlerin, özellikle IgAV olmak üzere sistemik vaskülitlerin bir komponenti olma olasılığı yüksektir. LCV'li yetişkinlerin çoğu komorbiditelere sahiptir ve kortikosteroidlere ihtiyaç duyar. Klinik uygulamada, DIF testinin LCV'li hastaları sınıflandırmadaki etkisi sınırlıdır.

References

  • Caproni M, Verdelli A. An update on the nomenclature for cutaneous vasculitis. Curr Opin Rheumatol. 2019;31(1):46-52. doi:10.1097/BOR. 0000000000000563
  • Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. doi:10.1002/art.37715
  • Loricera J, Blanco R, Ortiz-Sanjuán F, et al. Single-organ cutaneous small-vessel vasculitis according to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides: a study of 60 patients from a series of 766 cutaneous vasculitis cases. Rheumatology (Oxford). 2015;54(1):77-82. doi:10.1093/rheumatology/keu295
  • Bodakçi E. Clinical study on single-organ cutaneous small vessels vasculitis: a retrospective observational study. Eur Rev Med Pharmacol Sci. 2024;28(2):534-541. doi:10.26355/eurrev_202401_35051
  • Davarci E, Afşar FŞ, Özkavruk Eliyatkın N. Evaluation of clinicopathological characteristics in single-organ cutaneous small vessel vasculitis. Güncel Tıbbi Araştırmaları Dergisi. 2025;5(1):22-36.
  • Sunderkötter CH, Zelger B, Chen KR, et al. Nomenclature of cutaneous vasculitis: dermatologic addendum to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.Arthritis Rheumatol. 2018;70(2):171-184. doi:10.1002/art.40375
  • Jessop SJ. Cutaneous leucocytoclastic vasculitis: a clinical and aetiological study. Br J Rheumatol. 1995;34(10):942-945. doi:10.1093/rheumatology/34.10.942
  • Johnson EF, Wetter DA, Lehman JS, Hand JL, Davis DMR, Tollefson MM. Leukocytoclastic vasculitis in children: clinical characteristics, subtypes, causes and direct immunofluorescence findings of 56 biopsy-confirmed cases. J Eur Acad Dermatol Venereol. 2017;31(3):544-549. doi: 10.1111/jdv.13952
  • Piram M, Mahr A. Epidemiology of immunoglobulin A vasculitis (Henoch-Schönlein): current state of knowledge. Curr Opin Rheumatol. 2013;25(2):171-178. doi:10.1097/BOR.0b013e32835d8e2a
  • Gupta S, Handa S, Kanwar AJ, Radotra BD, Minz RW. Cutaneous vasculitides: clinico-pathological correlation. Indian J Dermatol Venereol Leprol. 2009;75(4):356–62
  • Ruperto N, Ozen S, Pistorio A, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: overall methodology and clinical characterisation. Ann Rheum Dis. 2010;69(5):790-797. doi:10.1136/ard.2009.116624
  • Sais G, Vidaller A, Jucglà A, Servitje O, Condom E, Peyri J. Prognostic factors in leukocytoclastic vasculitis: a clinicopathologic study of 160 patients. Arch Dermatol. 1998;134(3):309-315. doi:10.1001/archderm. 134.3.309
  • Bouiller K, Audia S, Devilliers H, et al. Etiologies and prognostic factors of leukocytoclastic vasculitis with skin involvement: a retrospective study in 112 patients. Medicine (Baltimore). 2016;95(28):e4238. doi:10. 1097/MD.0000000000004238
  • García-Porrúa C, González-Gay MA. Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch-Schönlein purpura in adults. Semin Arthritis Rheum. 1999;28(6):404-412. doi:10.1016/s0049-0172(99)80006-7
  • Pastuszczak M, Celińska-Löwenhoff M, Sułowicz J, Wojas-Pelc A, Musiał J. Clinical study on single-organ cutaneous small vessels vasculitis (SoCSVV). Medicine (Baltimore). 2017;96(12):e6376. doi:10. 1097/MD.0000000000006376
  • Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M. Cutaneous vasculitis in children and adults. Associated diseases and etiologic factors in 303 patients. Medicine (Baltimore). 1998;77(6):403-418. doi:10.1097/00005792-199811000-00007
  • Villatoro-Villar M, Crowson CS, Warrington KJ, Makol A, Ytterberg SR, Koster MJ. Clinical characteristics of biopsy-proven IgA vasculitis in children and adults: a retrospective cohort study. Mayo Clin Proc. 2019;94(9):1769-1780. doi:10.1016/j.mayocp.2019.04.034
  • Nossent J, Raymond W, Isobel Keen H, Preen D, Inderjeeth C. Morbidity and mortality in adult-onset IgA vasculitis: a long-term population-based cohort study. Rheumatology (Oxford). 2021;61(1):291-298. doi:10.1093/rheumatology/keab312
  • Boesen EI, Kakalij RM. Autoimmune-mediated renal disease and hypertension. Clin Sci (Lond). 2021;135(17):2165-2196. doi:10.1042/CS 20200955
  • 20. Huang X, Wu X, Le W, et al. Renal prognosis and related risk factors for Henoch-Schönlein purpura nephritis: a chinese adult patient cohort. Sci Rep. 2018;8(1):5585. doi:10.1038/s41598-018-23638-2
  • Kaynar Özçelik G, Içaçan OC, Yalçin Mutlu M, Yildirim F, Bes C. Palpable purpura: is it associated with vasculitis or not? A single-center experience. Clin Rheumatol. 2022;41(5):1493-1498. doi:10.1007/s10067-022-06075-5
  • Younger DS, Carlson A. Dermatologic aspects of systemic vasculitis.Neurol Clin. 2019;37(2):465-473. doi:10.1016/j.ncl.2019.01.017
  • Fraticelli P, Benfaremo D, Gabrielli A. Diagnosis and management of leukocytoclastic vasculitis. Intern Emerg Med. 2021;16(4):831-841. doi: 10.1007/s11739-021-02688-x
  • Arora A, Wetter DA, Gonzalez-Santiago TM, Davis MDP, Lohse CM. Incidence of leukocytoclastic vasculitis, 1996 to 2010: a population-based study in Olmsted County, Minnesota. Mayo Clin Proc. 2014;89(11): 1515-1524. doi:10.1016/j.mayocp.2014.04.015
  • Feasel P, Billings SD, Bergfeld WF, Piliang MP, Fernandez AP, Ko JS. Direct immunofluorescence testing in vasculitis-a single institution experience with Henoch-Schönlein purpura. J Cutan Pathol. 2018;45(1): 16-22. doi:10.1111/cup.13054
  • Nandeesh B, Tirumalae R. Direct immunofluorescence in cutaneous vasculitis: experience from a referral hospital in India. Indian J Dermatol. 2013;58(1):22-25. doi:10.4103/0019-5154.105280
  • Frumholtz L, Laurent-Roussel S, Lipsker D, Terrier B. Cutaneous vasculitis: review on diagnosis and clinicopathologic correlations. Clin Rev Allergy Immunol. 2021;61(2):181-193. doi:10.1007/s12016-020-08788-4
  • Lath K, Chatterjee D, Saikia UN, et al. Role of direct immunofluorescence in cutaneous small-vessel vasculitis: experience from a tertiary center. Am J Dermatopathol. 2018;40(9):661-666. doi:10.1097/DAD. 0000000000001170
  • Barnadas MA, Pérez E, Gich I, et al. Diagnostic, prognostic and pathogenic value of the direct immunofluorescence test in cutaneous leukocytoclastic vasculitis. Int J Dermatol. 2004;43(1):19-26. doi:10.1111/j.1365-4632.2004.01714.x
  • Alalwani M, Billings SD, Gota CE. Clinical significance of immunoglobulin deposition in leukocytoclastic vasculitis: a 5-year retrospective study of 88 patients at cleveland clinic. Am J Dermatopathol. 2014;36(9):723-729. doi:10.1097/DAD.0000000000000122
  • Lehman JS, Ferringer TC, Fung MA, Cassarino DS, Shalin SC. Diagnostic utility of direct immunofluorescence test panels for cutaneous vasculitis: a scoping review. J Cutan Pathol. 2024;51(12):987-999. doi:10.1111/cup.14722
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Details

Primary Language English
Subjects Rheumatology and Arthritis
Journal Section Research Articles
Authors

Fatih Yıldırım 0000-0003-3909-7500

Şevket Ali Ekmen 0000-0003-4393-5319

Cemal Bes 0000-0002-1730-2991

Publication Date July 28, 2025
Submission Date June 30, 2025
Acceptance Date July 14, 2025
Published in Issue Year 2025 Volume: 7 Issue: 4

Cite

AMA Yıldırım F, Ekmen ŞA, Bes C. Clinical and pathological evaluation of patients with leukocytoclastic vasculitis: a retrospective single-center rheumatology experience. Anatolian Curr Med J / ACMJ / acmj. July 2025;7(4):485-491. doi:10.38053/acmj.1731053

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