PEMBE OLTULU, Ayşenur UĞUR, İLKAY ÖZER, FAHRİYE KILINÇ, HACI HASAN ESEN, Sıdıka FINDIK, ARZU ATASEVEN, MEHMET SİNAN İYİSOY, MUSTAFA CİHAT AVUNDUK, Şükrü BALEVİ

Kutanöz Küçük Damar Vaskülitlerinde Direkt İmmunfloresan (Dif) Mikroskopi Bulguları: Tek Merkeze Ait Deneyimler

Giriş: Küçük damar kutanöz vaskülitlerinde (KKV) direkt immunfloresanmikroskopide (DİF) immun depolanmalar (özellikleIgG, IgM, IgA ve Kompleman C3) gözlenebilir. Dünya literatüründeçeşitli pozitiflik oranları bildiren çalışmalar mevcuttur. Çalışmamızdakliniğimize ait KKV’lerinin DİF mikroskopi sonuçlarını sunmayıamaçladık. Gereç ve yöntem: Vaskülit ön tanısı ile biopsi ve DİF tetkikiyapılan, histopatolojik olarak KKV’i mevcut olan toplam 121olgu retrospektif olarak çalışmaya dahil edildi. Olgular klinik verilerive Chapel Hill Consensus Conference vaskülit sınıflaması gözönündebulundurularak toplam 6 gruba ayrıldı. Bazal membran ya da perivasküler(PV) alanda en az bir depolanma ‘DİF pozitif’ olarak kabuledildi. Tüm olgularda DİF IgG, IgM, IgA ve Compleman C3 depolanmalarınındağılımları, oranları ve gruplarda en az bir immun depozitinbulunma durumu belirlendi. Lökositoklazis bulunduran ya daeozinofil bulunduranlar ayrı grup yapılarak diğerleri ile immun depolanmalaraçısından istatistiksel olarak karşılaştırıldı. Bulgular: Tümolgularda DİF pozitifliği %58.7 (n:71/121) idi. Lökositoklastik vaskülitolgularının %50.9’unda (n:28/55), nonspesifik KV olgularının%67.4’ünde (n:31/46), ürtikeryal vaskülit olgularının %44.4(n:4/9)’ünde, livedoid vaskülit olgularının %75 (n:3/4)’inde, henochschonlein purpurası (HSP) olgularının (n:5/5) %100’ünde DİF pozitifti.2 vaskülopati olgusunda depolanma yoktu. Lökositoklazis veeozinofil mevcudiyeti ile immun depolanmalar arasında herhangi birilişki yoktu. En fazla biriken depozit C3 iken, HSP olgularında IgAdepolanma oranı %100’dü. Sonuç: KKV’lerinde gözardı edilemeyecekyüksek DİF pozitiflik oranları (özellikle C3) tespit edildi. HSPolgularında DİF ile IgA depozit tespiti tanı için oldukça önemlidir.KKV’lerde DİF tetkikinin, klinik ve histopatolojik incelemeye ekolarak uygulanması faydalı olabilir.

Direct Immunofluorescence (Dif) Microscopy In Cutanous Small Vessel Vasculitis: A Single Institution Experiences

Background: İmmun deposits (especially IgG, IgM, IgA and Compleman C3) can be observed under direct immunofluorescence microscopy (DIF) in many patients with cutaneous small vessel vasculitis (CSV). There are some studies reporting various positivity rates in the literature. İn this study, we aimed to present the results of DIF microscopy of CSV cases in our ınstitute. Materyal and Methods: A total of 121 patients with CSV were included retrospectively in this study. All the cases have skin biopsy for DIF and histopathological examination and clinical data. A total of 6 groups were formed considering the clinical data of the cases and the Chapel Hill Consensus Conference vasculitis classification. The accumulation of at least one of the immun deposits (IgG, IgM, IgA ve C3) in basal membrane or perivascular (PV) field was assessed as ‘DIF positive’. The relationship between the presence of eosinophil and leucocytoclasia and the accumulation of immun deposits was investigated statistically. Results: DIF was positive in 58.7% (n:71/121) cases. DIF was positive in 50.9% (n: 28/55) of leukocytoclastic vasculitis cases, 67.4% (n: 31/46) of nonspecific CV cases, 44.4% (n: 4/9) of urticarial vasculitis cases and 75% of livedoid vasculitis cases (n: 3/4), 100% (n: 5/5) of henoch schonlein purpura (HSP) cases. There was not any accumulation in vasculopathy cases (n:2). There was not any relationship statistically between the presence of eosinophil and leucocytoclasia and the accumulation of immun deposits. The highest immun accumulation was C3 and the rate of IgA accumulation in HSP was 100%. Conclusion: In CSVs, high DIF positivity rates (especially C3) were determined. The determination of IgA deposits with DIF is very important for diagnosis of HSP. Performing of DIF in addition to clinical and histopathologic examination may be useful in CSVs.

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Kavala M, Zindancı İ. The Management of Pemphigus Patients: Clinical Remission, Discontinuation of Therapy and Our Experience. Türkderm. 2008;42(1):13-14.
Lebe B, Gül Nıflıoğlu G, Seyrek S, Ellıdokuz H. Evaluation of clinical and histopathologic/ direct immunofluorescence diagnosis in au-toimmune vesiculobullous dermatitis: utility of direct immunofluorescence. Turk Patoloji Derg 2012;28(1):11-16.
Şahin EB, Hapa A, Elçin G, Karaduman A, Ersoy Evans S, Erkin G, et al. Lökositoklastik Vaskülit: 60 Hastanın Geriye Dönük Analizi. Turk J Dermatol 2011;5:85-91.
Niflioğlu GG, Lebe B. Pemphigoid Gestationis: Light Microscopic and Direct Immunofluorescense Findings. Turk Patoloji Derg. 2014;30(2):152-154.
Arslan Z, Özmen S, Sürmeli S, Arda N. Atypical acute urticaria in children and its relationship with urticarial vasculitis. Turk J Med Sci 2011;41(1):87-92.
Poterucha TJ, Wetter DA, Gibson LE, Camilleri MJ, Lohse CM. Correlates of systemic disease in adult Henoch-Schönlein purpura: a retrospective study of direct immunofluorescence and skin lesion distribution in 87 patients at Mayo Clinic. J Am Acad Dermatol 2012;67 (4):612-616.
Valencia-Guerrero A, Deng A, Dresser K, Bouliane G, Cornejo KM. The Value of Direct Immunofluorescence on Proteinase-Digested Formalin-Fixed Paraffin-Embedded Skin Biopsies. Am J Dermatopathol. 2018;40(2):111-117.
Otten JV, Hashimoto T, Hertl M, Payne AS, Sitaru C. Molecular diagnosis in autoimmune skin blistering conditions. Curr Mol Med 2014;14(1):69-95.
İlter N, Adışen E. Kutanöz vaskülitler. Türkderm 2010;44:50-60.
Linskey KR, Kroshinsky D, Mihm MC Jr, Hoang MP. Immunoglobulin-A-associated small-vessel vasculitis: a 10-year experience at the Massachusetts General Hospital. Acad Dermatol. 2012;66(5):813-822.
Sais G, Vidaller A. Role of direct immunofluorescence test in cutaneous leukocytoclastic vasculitis. Int J Dermatol. 2005;44(11):970- 971.
Poornimambaa M1, Asokan N1, Augustine J. Utility of Direct Immunofluorescence in the Diagnosis of Small Vessel Vasculitis of the Skin: A Cross-Sectional Study. Indian Dermatol Online J. 2017;8(6):515-517.
Mysorekar VV, Sumathy TK, Shyam Prasad AL. Role of direct immunofluorescence in dermatological disorders. Indian Dermatol Online J 2015;6(3):172-180.
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.
Takatu CM, Heringer APR, Aoki V, Valente NYS, de Faria Sanchez PC, de Carvalho JF, 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.
Nandeesh B, Tirumalae R. Direct immunofluorescence in cutaneous vasculitis: experience from a referral hospital in India. Indian J Dermatol 2013;58(1):22-25.
Elmacı AM, Özekinci S, Baran A.Dokuz Yaşında Bir ANCA İlişkili Vaskülit. Selçuk Tıp Derg 2014;30 Ek Sayı-1:31-33.
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.
Gonzalez-Gay MA, Garcia-Porrua C, Salvarani C, Lo Scocco G, Pujol RM. Cutaneous vasculitis: a diagnostic approach. Clin Exp Rheumatol 2003;21(6 Suppl 32):85-88.
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised Internatio-nal Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11.
Carlson JA. The histological assessment of cutaneous vasculitis. Histopathology. 2010;56 (1):3-23.
McLaren JS, McRorie ER, Luqmani RA. Diagnosis and assessment of systemic vasculitis. Clin Exp Rheumatol. 2002;20(6):854-862.
Carlson JA, Ng BT, Chen KR. Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. Am. J. Dermatopathol 2005;27(6):504–528.