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Tek-organ kutanöz küçük damar vaskülitinin klinikopatolojik özelliklerinin değerlendirilmesi

Year 2025, Volume: 5 Issue: 1, 22 - 36, 30.04.2025

Erkan Davarci , Fatma Şule Afşar , Nuket Özkavruk Eliyatkın

Abstract

Amaç: Kutanöz vaskülit, deri kan damarlarının enflamasyonu ile karakterize geniş ve heterojen bir vaskülitik sendrom spek-trumunu içerir. Hastalık deriyle sınırlı olduğunda, tek organlı kutanöz küçük damar vasküliti terimi kullanılır. Bu antiteye ilişkin veriler sınırlıdır. Bu çalışmada tek organlı kutanöz küçük damar vasküliti tanısı alan hastaların klinikopatolojik özelliklerinin retrospektif olarak değerlendirilmesi amaçlanmıştır.
Gereç ve Yöntemler:Yüz sekiz hasta çalışmaya dahil edildi. Hastaların demografik özellikleri, klinik özellikleri, laboratuvar bul-guları, etiyolojik faktörleri, tedavi modaliteleri ve prognozları değerlendirildi. Hastaların biyopsi örnekleri histopatolojik olarak yeniden değerlendirildi.
Bulgular: Hastaların %82,4’ünde akral tutulum mevcuttu. Palpabl purpura (%82,4) en sık görülen deri lezyonuydu. Klinik prez-entasyon 80 (%74,1) hastada akut iken, 24 (%23,1) hastada nüks görüldü. Kronik hastalarda nüks ve ülseratif lezyonlar daha sık gözlendi. Nüks eden hastalarda yaş ortalaması daha düşük bulundu. Hastaların %75’inde etiyoloji idiyopatikti. CRP yüksekliği şiddetli fibrinoid nekroz ve subkutan doku tutulumu olan hastalarda anlamlıydı. Topikal kortikosteroidler ve nonsteroid anti-inflamatuvar ilaçlarla tedavi seçeneği (%54,3) en yaygın tipti. Klinik ve histopatolojik özelliklerin nüks üzerinde etkisi olmadığı, ancak tedavi tipinin nüks ile ilişkili olduğu bulunmuştur.
Sonuç:Tek organ kutanöz küçük damar vasküliti ile ilgili az sayıda çalışma vardır. Sonuçlar diğer çalışmaların özelliklerine ben-zerdi. Klinik ve histopatolojik özelliklerin birlikte değerlendirileceği ileri çalışmalara ihtiyaç vardır

Keywords

Tek-organ kutanöz küçük damar vasküliti Klinik özellikler Histopatoloji

Ethical Statement

The study was approved by the Ethics Committee of Izmir Ataturk Training and Research Hospital and adhered to the principles of the Declaration of Helsinki (2020GOKAE-0374) on 17 September 2020

Project Number

2020GOKAE-0374

References

  • Frumholtz L., Laurent-Roussel S., Lipsker D., Terrier B. Cutaneous vasculitis: review on diagnosis and clinicopathologic correlations. Clin Rev Allergy Immunol 2021; 61(2):181-93. Doi: 10.1007/ s12016-020-08788-4.
  • 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.
  • Bodakçi E. Clinical study on single-organ cutaneous small vessels vasculitis: a retrospective observational study. Eur Rev Med Pharmacol Sci 2024; 28(2):534-41. Doi: 10.26355/eurrev_ 202401_35051.
  • Morita TCAB., Criado PR., Criado RFJ., Trés GFS., Sotto MN. Update on vasculitis: overview and relevant dermatological aspects for the clinical and histopathological diagnosis - Part II. An Bras Dermatol 2020; 95(4):493-507. Doi: 10.1016/j. abd.2020.04.004.
  • Gambichler T., Ardabili L., Domin B., Susok L., Rached NA. Predictors of disease severity, length of hospitalization, and recurrence in inpatients with single-organ cutaneous small vessel vasculitis. Eur J Dermatol 2024; 34(4):378-83. Doi: 10.1684/ejd.2024.4723.
  • 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.
  • 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):171-84. Doi: 10.1002/art.40375.
  • 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.
  • 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.
  • Cakiter AU., Kucuk OS., Ozkaya DB., Topukcu B., Onsun N. Demographic characteristics, aetiology, and assessment of treatment options in leukocytoclastic vasculitis. Postepy Dermatol Alergol 2017; 34(2):104-9. Doi: 10.5114/ ada.2017.67071.
  • Carlson JA., Ng BT., Chen KR. Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. Am J Dermatopathol 2005; 27(6):504- 28. Doi: 10.1097/01.dad.0000181109.54532. c5.
  • Ekenstam Eaf., Callen JP. Cutaneous leukocytoclastic vasculitis. Clinical and laboratory features of 82 patients seen in private practice. Arch Dermatol 1984; 120(4):484-9. Doi: 10.1001/archderm. 120.4.484.
  • Morita TCAB., Trés GFS., Criado RFJ., Sotto MN., Criado PR. Update on vasculitis: an overview and dermatological clues for clinical and histopathological diagnosis - part I. An Bras Dermatol 2020; 95(3):355-71. Doi: 10.1016/j.abd.2020.01.003.
  • Shavit E., Alavi A., Sibbald RG. Vasculitis-what do we have to know? A review of literature. Int J Low Extrem Wounds 2018; 17(4):218-26. Doi: 10.1177/1534734618804982.
  • Cupps TR., Springer RM., Fauci AS. Chronic, recurrent small-vessel cutaneous vasculitis. Clinical experience in 13 patients. JAMA 1982; 247(14):1994-8. PMID: 7062504.
  • Takatu CM., Heringer APR., Aoki V., et al. Clinicopathologic correlation of 282 leukocytoclastic vasculitis cases in a tertiary hospital: a focus on direct immunofluorescence findings at the blood vessel wall. Immunol Res 2017; 65(1):395-401. Doi: 10.1007/s12026-016-8850-6.
  • Latha S., Choon SE., Tey KE., Chee YN. Clinical features and prognostic factors of cutaneous vasculitis among dermatology patients in Johor Bahru, Malaysia. Med J Malaysia 2017; 72(6):345-9. PMID: 29308771.
  • Jennette JC., Falk RJ., Andrassy K., et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37(2):187-92. Doi: 10.1002/ art.1780370206.
  • Swamy SM., Bhat RM RB. Cutaneous vasculitis : An etiological and clinicopathological study. International Journal of Scientific and Research Publications 2015; 5(9):1-6.
  • Khetan P., Sethuraman G., Khaitan BK, et al. An aetiological & clinicopathological study on cutaneous vasculitis. Indian J Med Res 2012; 135(1):107-13. Doi: 10.4103/09715916.93432.
  • Tai YJ., Chong AH., Williams RA., Cumming S., Kelly RI. Retrospective analysis of adult patients with cutaneous leukocytoclastic vasculitis. Australas J Dermatol 2006; 47(2):92-6. Doi: 10.1111/j.1440-0960.2006.00239.x.
  • 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):309-15. Doi: 10.1001/archderm.134.3.309.

Evaluation of clinicopathological characteristics in single-organ cutaneous small vessel vasculitis

Year 2025, Volume: 5 Issue: 1, 22 - 36, 30.04.2025

Erkan Davarci , Fatma Şule Afşar , Nuket Özkavruk Eliyatkın

Abstract

Objective: Cutaneous vasculitis includes a wide and heterogeneous spectrum of vasculitic syndromes characterized by inflam-mation of skin blood vessels. When the disease is confined to the skin, the term single-organ cutaneous small vessel vasculitis is used. Data regarding this entity is limited. This study aims to retrospectively evaluate the clinicopathological characteristics of patients who were diagnosed with single-organ cutaneous small vessel vasculitis retrospectively.
Material and Methods: One hundred eight patients were included in the study. Demographic characteristics, clinical features, laboratory findings, etiological factors, treatment modalities, and prognoses of 108 patients were evaluated. Biopsy speci-mens of patients were re-evaluated histopathologically.
Results: Most frequently acral involvement, and palpable purpura were observed in 82.4% of the patients. Clinical presentation was acute in 80 (74.1%) patients and relapse was seen in 24 (23.1%) patients. Relapse and ulcerative lesions were observed more frequently in chronic patients. The mean age was found to be lower in relapsing patients. Etiology was idiopathic in 75% of the patients. The elevation of CRP was significant in patients with severe fibrinoid necrosis and subcutaneous tissue involvement. Treatment option with topical corticosteroids and nonsteroidal anti-inflammatory drugs (54,3%) was the most common type. The clinical and histopathological features were found to have no effect on relapse, but treatment type was associated with relapse.
Conclusion: There are few studies on single organ cutaneous small vessel vasculitis. Results were similar to the features of the other studies. Further studies in which clinical and histopathological features will be evaluated together are needed.

Keywords

Single-organ cutaneous small vessel vasculitis Clinical features Histopathology

Project Number

2020GOKAE-0374

References

  • Frumholtz L., Laurent-Roussel S., Lipsker D., Terrier B. Cutaneous vasculitis: review on diagnosis and clinicopathologic correlations. Clin Rev Allergy Immunol 2021; 61(2):181-93. Doi: 10.1007/ s12016-020-08788-4.
  • 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.
  • Bodakçi E. Clinical study on single-organ cutaneous small vessels vasculitis: a retrospective observational study. Eur Rev Med Pharmacol Sci 2024; 28(2):534-41. Doi: 10.26355/eurrev_ 202401_35051.
  • Morita TCAB., Criado PR., Criado RFJ., Trés GFS., Sotto MN. Update on vasculitis: overview and relevant dermatological aspects for the clinical and histopathological diagnosis - Part II. An Bras Dermatol 2020; 95(4):493-507. Doi: 10.1016/j. abd.2020.04.004.
  • Gambichler T., Ardabili L., Domin B., Susok L., Rached NA. Predictors of disease severity, length of hospitalization, and recurrence in inpatients with single-organ cutaneous small vessel vasculitis. Eur J Dermatol 2024; 34(4):378-83. Doi: 10.1684/ejd.2024.4723.
  • 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.
  • 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):171-84. Doi: 10.1002/art.40375.
  • 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.
  • 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.
  • Cakiter AU., Kucuk OS., Ozkaya DB., Topukcu B., Onsun N. Demographic characteristics, aetiology, and assessment of treatment options in leukocytoclastic vasculitis. Postepy Dermatol Alergol 2017; 34(2):104-9. Doi: 10.5114/ ada.2017.67071.
  • Carlson JA., Ng BT., Chen KR. Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. Am J Dermatopathol 2005; 27(6):504- 28. Doi: 10.1097/01.dad.0000181109.54532. c5.
  • Ekenstam Eaf., Callen JP. Cutaneous leukocytoclastic vasculitis. Clinical and laboratory features of 82 patients seen in private practice. Arch Dermatol 1984; 120(4):484-9. Doi: 10.1001/archderm. 120.4.484.
  • Morita TCAB., Trés GFS., Criado RFJ., Sotto MN., Criado PR. Update on vasculitis: an overview and dermatological clues for clinical and histopathological diagnosis - part I. An Bras Dermatol 2020; 95(3):355-71. Doi: 10.1016/j.abd.2020.01.003.
  • Shavit E., Alavi A., Sibbald RG. Vasculitis-what do we have to know? A review of literature. Int J Low Extrem Wounds 2018; 17(4):218-26. Doi: 10.1177/1534734618804982.
  • Cupps TR., Springer RM., Fauci AS. Chronic, recurrent small-vessel cutaneous vasculitis. Clinical experience in 13 patients. JAMA 1982; 247(14):1994-8. PMID: 7062504.
  • Takatu CM., Heringer APR., Aoki V., et al. Clinicopathologic correlation of 282 leukocytoclastic vasculitis cases in a tertiary hospital: a focus on direct immunofluorescence findings at the blood vessel wall. Immunol Res 2017; 65(1):395-401. Doi: 10.1007/s12026-016-8850-6.
  • Latha S., Choon SE., Tey KE., Chee YN. Clinical features and prognostic factors of cutaneous vasculitis among dermatology patients in Johor Bahru, Malaysia. Med J Malaysia 2017; 72(6):345-9. PMID: 29308771.
  • Jennette JC., Falk RJ., Andrassy K., et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37(2):187-92. Doi: 10.1002/ art.1780370206.
  • Swamy SM., Bhat RM RB. Cutaneous vasculitis : An etiological and clinicopathological study. International Journal of Scientific and Research Publications 2015; 5(9):1-6.
  • Khetan P., Sethuraman G., Khaitan BK, et al. An aetiological & clinicopathological study on cutaneous vasculitis. Indian J Med Res 2012; 135(1):107-13. Doi: 10.4103/09715916.93432.
  • Tai YJ., Chong AH., Williams RA., Cumming S., Kelly RI. Retrospective analysis of adult patients with cutaneous leukocytoclastic vasculitis. Australas J Dermatol 2006; 47(2):92-6. Doi: 10.1111/j.1440-0960.2006.00239.x.
  • 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):309-15. Doi: 10.1001/archderm.134.3.309.
There are 22 citations in total.

Details

Primary Language English
Subjects Dermatology, Pathology
Journal Section Research Articles
Authors

Erkan Davarci 0000-0002-7156-0406

Fatma Şule Afşar 0000-0002-9103-2782

Nuket Özkavruk Eliyatkın 0000-0002-7784-5699

Project Number 2020GOKAE-0374
Publication Date April 30, 2025
Submission Date November 10, 2024
Acceptance Date April 14, 2025
Published in Issue Year 2025 Volume: 5 Issue: 1

Cite

APA Davarci, E., Afşar, F. Ş., & Özkavruk Eliyatkın, N. (2025). Evaluation of clinicopathological characteristics in single-organ cutaneous small vessel vasculitis. Güncel Tıbbi Araştırmaları Dergisi, 5(1), 22-36.
AMA Davarci E, Afşar FŞ, Özkavruk Eliyatkın N. Evaluation of clinicopathological characteristics in single-organ cutaneous small vessel vasculitis. CJMR. April 2025;5(1):22-36.
Chicago Davarci, Erkan, Fatma Şule Afşar, and Nuket Özkavruk Eliyatkın. “Evaluation of Clinicopathological Characteristics in Single-Organ Cutaneous Small Vessel Vasculitis”. Güncel Tıbbi Araştırmaları Dergisi 5, no. 1 (April 2025): 22-36.
EndNote Davarci E, Afşar FŞ, Özkavruk Eliyatkın N (April 1, 2025) Evaluation of clinicopathological characteristics in single-organ cutaneous small vessel vasculitis. Güncel Tıbbi Araştırmaları Dergisi 5 1 22–36.
IEEE E. Davarci, F. Ş. Afşar, and N. Özkavruk Eliyatkın, “Evaluation of clinicopathological characteristics in single-organ cutaneous small vessel vasculitis”, CJMR, vol. 5, no. 1, pp. 22–36, 2025.
ISNAD Davarci, Erkan et al. “Evaluation of Clinicopathological Characteristics in Single-Organ Cutaneous Small Vessel Vasculitis”. Güncel Tıbbi Araştırmaları Dergisi 5/1 (April 2025), 22-36.
JAMA Davarci E, Afşar FŞ, Özkavruk Eliyatkın N. Evaluation of clinicopathological characteristics in single-organ cutaneous small vessel vasculitis. CJMR. 2025;5:22–36.
MLA Davarci, Erkan et al. “Evaluation of Clinicopathological Characteristics in Single-Organ Cutaneous Small Vessel Vasculitis”. Güncel Tıbbi Araştırmaları Dergisi, vol. 5, no. 1, 2025, pp. 22-36.
Vancouver Davarci E, Afşar FŞ, Özkavruk Eliyatkın N. Evaluation of clinicopathological characteristics in single-organ cutaneous small vessel vasculitis. CJMR. 2025;5(1):22-36.
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