#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#

ANCA associated vasculitis with skin ulcers of the cald, clinically manifested as pyoderma gangrenosum


Authors: B. Průchová 1;  H. Štilcová 2;  M. Podhola 3;  D. Kacerovská 4;  T. Soukup 2
Authors‘ workplace: Oblastní nemocnice Náchod a. s., Interní oddělení Nemocnice Rychnov nad Kněžnou 1;  Subkatedra revmatologie, II. interní gastroenterologická klinika LF UK a FN, Hradec Králové 2;  Fingerlandův ústav patologie LF a FN, Hradec Králové 3;  Šiklův ústav patologie LF a FN, Plzeň 4
Published in: Čes. Revmatol., 29, 2021, No. 4, p. 228-236.
Category: Case Report

Overview

ANCA (antineutrophil cytoplasmic antibody) – associated vasculitis (AAV) is one of the chronic systemic inflammatory diseases of small and medium caliber vessels. The most commonly affected are the airways, where necrotizing granulomas form, and the kidneys in the form of focal segmental necrotizing glomerulonephritis with crescent formation. Diagnosis is based on clinical symptoms, detection of ANCA antibodies, and biopsy of the affected organ tissue. Pyoderma gangrenosum (PG) is a very rare chronic inflammatory skin disease with the formation of painful ulcers. The etiopathogenesis is not fully understood; it is probably immunopathology with neutrophil dysfunction. We determine the diagnosis on the basis of diagnostic criteria, which are based primarily on histopathological findings, a clinical picture with a rapid response to immunosuppressive therapy. In ⅔ of cases, it is associated with other systemic autoimmune diseases (inflammatory bowel diseases, arthritis, hematological malignancies, etc.)

We describe a case of a hitherto healthy 53-year-old woman who developed painful ulcerative defects of the right calf, followed by the development of phlegmon. Defects developed after a minor injury. Doppler ultrasonography of the arteries excluded the ischemic basis of the resulting defect, and the culture excluded the infectious etiology of the disease. Subsequent skin biopsy from a defect with numerous neutrophilic granulocytes found a diagnosis of PG. The diagnosis of ANCA vasculitis was made on the basis of positive ANCA antibodies (against type 3proteinase) and histological findings from a kidney biopsy.

The diagnosis of PG is rare, so it is necessary to think about it in non-healing ulcers. If the diagnosis is made, it is important to look for possible associated diseases. As in most cases, an association with other serious conditions has been demonstrated; in our case, ANCA necrotizing small-vessel vasculitis was found.

Keywords:

ANCA associated vasculitis – pyoderma gangrenosum – shin ulcers


Sources

1. Pavelka K. Klinická revmatologie. Praha: Galén 2018; 412–415.

2. Pavelka K. Klinická revmatologie. Praha: Galén 2018; 636.

3. Bečvář R, Tesař V. Vaskulitidy v klinické praxi – diagnostika a terapie. Praha: Medprint 1994; 94–103.

4. Martinková K, Valkovský I, Handlos P. Granulomatóza s polyangiitidou. Interní Med 2014; 16: 199–201.

5. Yates M, Watts A, Bajema IM, Cid MC, Crestani B, Hauser T, et al. Eular/ERA-EDTA recommendations for the management of ANCA associated vaskulitis. Ann Rheum Dis 2016; 75: 1583–1594.

6. Bečvář R. Nové trendy v monitorování a léčbě ANCA asociovaných vaskulitid. Vnitř. Lék. 2018; 64: 164–168.

7. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990; 33(8): 1101–1107.

8. Hrnčíř Zb, Sobotka L, Pospíšil M, Prášil P, Brtková J, Laco J. Pyoderma gangrenosum a nekrotizující arteritida u pozdně začí- nající revmatoidní artritidy. Čes. Revmatol. 2018; 26: 59–63.

9. Stefflová E. Pyoderma gangraenosum. Prakt. Lék. 2003; 9: 522– 523.

10. Jarčušková D, Benečová K, Ficová M, Jautová J. Pyoderma gangraenosum – klinické aspekty, liečba. Čes-slov. Derm. 2002; 80–83.

11. Zarraga M, Swenson N, Glick B. Pyoderma Gangrenosum- associated granulomatosis with Polyangitis: A Case Report and Literature Review. J Clin Aesthet Dermatol 2017; 10(10): 40–42.

12. Oakley A. Pyoderma gangrenosum. 2015, https://dermnetnz. org/topics/pyoderma-gangrenosum/

13. Gastman B, Hashem AM, Djohan R, Bernard S, Hendrickson M, Schwarz G, et al. Malignant pyoderma associated with granulomatosis with polyangiitis (Wegener granulomatosis) as a unique indication for facial vascularized composite allotransplantation. Plastic Reconstructive Surgery 2016; 137(6): 1007e–1015e.

14. Genovese G, Tavecchio S, Berti E, Rongioletti F, Marzano AV. Pyoderma gangrenosum-like ulcerations in granulomatosis with polyangiitis: two cases and literature Review. Rheumatol Int 2018; 38: 1139–1151.

15. Tashtoush B, Memarpour R, Johnston Y, Ramirez J. Large pyoderma gangrenosum-like ulcers: a rare presentation of granulomatosis with polyangiitis. Case Rep Rheumatol 2014; 14: 850364.

16. Jorgaqi E, Fida M, Janushaj E, Mala R, Vasili E. A rare case of granulomatous Wegener with multiple pyoderma gangrenosum-like ulcers. Dermatol Ther 2020; 33(2): e13255.

17. Moen BH, Nystad TW, Barrett TM, Sandvik LF. A boy in his teens with large ulcerations of the head and neck. Tidsskr Nor Laegeforen 2019; 8: 139.

18. Kass A, Fagan JD, Long P. Granulomatosis with Polyangiitis Presenting with Pyoderma Gangrenosum-Like Ulceration and Negative Cytoplasmic Antineutrophilic Cytoplasmic Antibodies in a Child. Pediatr Dermatol 2017; 34(5): e231–e234.

19. McConnell B, Said MS, Ramakrishnan VR. Nasal septal perforation associated with pyoderma gangrenosum. Allergy Rhinol (Providence) 2015; 6(2): 122–124.

20. Oz RS, Onajin O, Harel L, Tal R, Dallos T, Rosenblatt A, et al. Pyoderma gangrenosum-like ulceration as a presenting feature of pediatric granulomatosis with polyangiitis. Pediatr Rheumatol Online J 2021; 19(1): 81.

21. Donmez S, Pamuk ON, Gedik M, Recep AK, Bulut G. A case of granulomatosis with polyangiitis and pyoderma gangrenosum successfully treated with infliximab and rituximab. Int J Rheum Dis 2014; 17(4): 471–475.

22. Murthy RK, Jackson J, Chatham WW, Sami N. Extensive Pyoderma Gangrenosum Associated With Granulomatosis With Polyangiitis With Both Responsive to Rituximab. J Clin Rheumatol 2016; 22(7): 393–395.

23. Kindle SA, Camilleri MJ, Gibson LE, Davis MDP. Granulomatosis with polyangiitis mimicking classic inflammatory bowel disease-associated pyoderma gangrenosum. Int J Dermatol 2017; 56(1): e1–e3.

24. Gallo DJ, Planelles CA, Barrios ML, Hernández CR, Rodríguez AM, Moreno EG, et al. Pyoderma gangrenosum and Wegener granulomatosis-like syndrome induced by cocaine. Clin Exp Dermatol 2013; 38(8): 878–882.

25. Bittencourt MJS, Soares LFB, Lobato LS, Mançano AD, Leandro HSC, Fonseca DMD. Multiple cavitary pulmonary nodules in association with pyoderma gangrenosum: case report. An Bras Dermatol 2012; 87(2): 301–304.

26. Sinovich V, Snow J. Protracted superficial Wegener's granulomatosis. Australas J Dermatol 2003; 44(3): 207–214.

27. Hello CL, Bonte I, Mora JJ, Verneuil L, Noël LH, Guillevin L. Pyoderma gangrenosum associated with Wegener's granulomatosis: partial response to mycophenolate mofetil. Rheumatology (Oxford) 2002; 41(2): 236–237.

28. Szõcs HI, Torma K, Petrovicz E, Hársing J, Fekete G, Karpati S, et al. Wegener's granulomatosis presenting as pyoderma gangrenosum. Int J Dermatol 2003; 42(11): 898–902.

29. Kishore S, Mittal V, Ahuja S, Majithia V. When a wound is the harbinger of a serious underlying systemic illness. BMJ Case Rep 2018; 31: bcr2017222491.

30. Haun P, Wanchu A, Korcheva V, Fett N. A Preauricular Ulcer in a Patient With Conjunctival Injection and Persistent Lacrimation: Challenge. Am J Dermatopathol 2016; 38(3): 211, 244–245.

Labels
Dermatology & STDs Paediatric rheumatology Rheumatology

Article was published in

Czech Rheumatology

Issue 4

2021 Issue 4

Most read in this issue
Topics Journals
Login
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account

#ADS_BOTTOM_SCRIPTS#