Büllöz Pemfigoidde Deri Biyopsisinde Eozinofil Sayısının Prognostik Önemi

Öz

Büllöz pemfigoid, en yaygın vezikülobüllöz otoimmün hastalıktır ve hastaların yarısında klinik olarak periferik eozinofili görülür. Bu çalışmada, doku eozinofil sayısı ile periferik eozinofili, mukozal tutulum, hastalık şiddeti ve tedavi yanıtı arasındaki ilişkinin analiz edilmesi amaçlandı. Yetmiş yedi büllöz pemfigoid hastasına ait deri biyopsileri yeniden değerlendirildi. Doku eozinofil sayısına göre hastalar düşük (1–20 eozinofil/BBA) ve yüksek (>20 eozinofil/BBA) eozinofil sayısına sahip olgular olarak sınıflandırıldı. Periferik kan eozinofil sayısı, tedavi yanıtı gibi diğer bilgiler de kaydedildi. Hastaların yaş ortalaması 75,08 yıl (36–95) olup %51,9’u kadındı. Doku eozinofil sayısı 1 ile 167 arasında değişmekteydi ve ortalama 28,79 idi. Hastaların %39’unda periferik eozinofili izlendi. Periferik eozinofili ile doku eozinofil sayısı arasında anlamlı ilişki saptandı (p<0,001). Yüksek periferik eozinofili grubunda hastalık yaygınlığı istatistiksel olarak anlamlı düzeyde daha fazlaydı (p=0,034) ve periferik eozinofilisi olan olgularda tam remisyon oranı daha yüksekti (p=0,027). Doku eozinofil sayısı ile Sedimentasyon, CRP, mukozal tutulum, lezyon şiddeti ve tedavi yanıtı arasında anlamlı bir fark saptanmadı. Doku eozinofil sayısı ile periferik eozinofili yakından ilişkilidir. Periferik eozinofili, hastalık şiddeti ve tedavi yanıtı ile ilişkili bulunurken, doku eozinofil sayısının klinik özelliklerle korelasyonu saptanmadı. Literatürde farklı sonuçlar bildirilmektedir. Yüksek komorbidite ve ileri yaş ile hastalık kontrolünün zorlaşması ve tedavinin standardize edilememesinin bu duruma yol açtığı düşünülmektedir.

Anahtar Kelimeler

• Büllöz Pemfigoid
• doku eozinofil sayısı
• periferik eozinofili
• hastalık şiddeti

Prognostic Significance of Skin Eosinophil Counts in Bullous Pemphigoid

Öz

Bullous pemphigoid is the most common vesiculobullous autoimmune disease, and clinically, peripheral eosinophilia is observed in half of the patients. This study aimed to analyze the relationship between tissue eosinophil count and peripheral eosinophilia, mucosal involvement, disease severity, and treatment response. Skin biopsies from seventy-seven patients diagnosed with bullous pemphigoid were re-evaluated. Patients were classified according to tissue eosinophil count as having low (1–20 eosinophils/HPF) or high (>20 eosinophils/HPF) eosinophil counts. Additional data, such as peripheral blood eosinophil count and treatment response, were also recorded. The mean age of the patients was 75.08 years (range: 36–95), and 51.9% were female. Tissue eosinophil counts ranged from 1 to 167, with a mean of 28.79. Peripheral eosinophilia was detected in 39% of the patients. A significant correlation was found between peripheral eosinophilia and tissue eosinophil count (p<0.001). The high peripheral eosinophilia group had significantly more extensive disease (p=0.034), and patients with peripheral eosinophilia had a higher rate of complete remission (p=0.027). No significant association was found between tissue eosinophil count and ESR, CRP, mucosal involvement, lesion severity, or treatment response. Tissue eosinophil count and peripheral eosinophilia are closely related. While peripheral eosinophilia was associated with disease severity and treatment response, tissue eosinophil count was not correlated with clinical features. Different results have been reported in the literature. High comorbidity and advanced age, combined with difficulties in disease control and lack of standardized treatment protocols, are believed to contribute to the variability observed across studies.

Anahtar Kelimeler

• Bullous Pemphigoid
• tissue eosinophil count
• peripheral eosinophilia
• severity of disease

Kaynakça

1. 1. Lever WF. Pemphigus and Pemphigoid. Springfield, IL: Charles C. Thomas, 1965.
2. 2. Goldblum JR, Lamps LW, McKenney JK, Myers JL, Ackerman L V, Rosai JTATT. Rosai and Ackerman’s surgical pathology. 11th edition. Philadelphia, PA: Elsevier Philadelphia, PA; 2018.
3. 3. Uzun S, Durdu M, Akman A, Gunasti S, Uslular C, Memisoglu HR, et al. Pemphigus in the Mediterranean region of Turkey: A study of 148 cases. International Journal of Dermatology. 2006 May 7;45(5):523–8.
4. 4. Lu L, Chen L, Xu Y, Liu A. Global incidence and prevalence of bullous pemphigoid: A systematic review and meta‐analysis. Journal of Cosmetic Dermatology. 2022 Oct;21(10):4818–35.
5. 5. Kutlubay Z, Sevim Keçici A, Çelik U, Mat MC. A survey of bullous diseases in a Turkish university hospital: clinicoepidemiological characteristics and follow-up. Turkish journal of medical sciences. 2021 Feb 26;51(1):124–33.
6. 6. Patterson JW. Weedon’s Skin Pathology. 5th ed. Elsevier; 2021.
7. 7. Feliciani C, Joly P, Jonkman MF, Zambruno G, Zillikens D, Ioannides D, et al. Management of bullous pemphigoid: the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology. British Journal of Dermatology. 2015 Apr;172(4):867–77.
8. 8. Amber KT, Murrell DF, Schmidt E, Joly P, Borradori L. Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management. Clinical Reviews in Allergy & Immunology. 2018 Feb 4;54(1):26–51.

9. 9. Garrido PM, Aguado-Lobo M, Espinosa-Lara P, Soares-Almeida L, Filipe P. Association of Peripheral Blood and Cutaneous Eosinophils With Bullous Pemphigoid Disease Severity and Treatment Outcomes. Actas Dermo-Sifiliográficas. 2022 Oct;113(9):881–7.
10. 10. Gore Karaali M, Koku Aksu AE, Cin M, Leblebici C, Kara Polat A, Gurel MS. Tissue eosinophil levels as a marker of disease severity in bullous pemphigoid. Australasian Journal of Dermatology. 2021 May 22;62(2).
11. 11. Kridin K. Peripheral eosinophilia in bullous pemphigoid: prevalence and influence on the clinical manifestation. British Journal of Dermatology. 2018 Nov;179(5):1141–7.
12. 12. Farnaghi F, Ehsani A hooshang, Kamyab-Hesary K, Abbasian S, Seirafi H, Nasimi M. Correlation of dermal and blood eosinophilia with bullous pemphigoid disease severity. International Journal of Women’s Dermatology. 2020 Jun;6(3):171–5.
13. 13. Bernard P, Venot J, Constant F, Bonnetblane JM. Blood eosinophilia as a severity marker for bullous pemphigoid. Journal of the American Academy of Dermatology. 1987 Apr;16(4):879–81.
14. 14. Bushkell LL, Jordon RE. Bullous pemphigoid: A cause of peripheral blood eosinophilia. Journal of the American Academy of Dermatology. 1983 May;8(5):648–51.
15. 15. Baum S, Engler Markowitz M, Lyakhovitsky A, Gershon R, Aviv H, Segal Z, et al. Skin Eosinophil Counts in Bullous Pemphigoid as a Prognostic Factor for Disease Severity and Treatment Response. Acta Dermato-Venereologica. 2023 Jan 10;103:adv00850.
16. 16. Simon D, Borradori L, Simon H. Eosinophils as putative therapeutic targets in bullous pemphigoid. Experimental Dermatology. 2017 Dec 26;26(12):1187–92.
17. 17. Messingham KN, Crowe TP, Fairley JA. The Intersection of IgE Autoantibodies and Eosinophilia in the Pathogenesis of Bullous Pemphigoid. Frontiers in Immunology. 2019 Oct 4;10.
18. 18. Ishiura N, Fujimoto M, Watanabe R, Nakashima H, Kuwano Y, Yazawa N, et al. Serum levels of IgE anti-BP180 and anti-BP230 autoantibodies in patients with bullous pemphigoid. Journal of Dermatological Science. 2008 Feb;49(2):153–61.