Velsov sindrom udružen sa eozinofilnom granulomatozom sa poliangiitisom
Sažetak
Uvod. Velsov sindom (eozinofilni celulitis) je hronično rekurentno oboljenje koje odlikuju epizode eritematoznih i edematoznih plakova ili nodusa, uz povremenu pojavu hemoragičnih bula na trupu i ekstremitetima. Eozinofilna granulomatoza sa poliangiitisom, u ranijoj terminologiji Čarg-Štrausov sindrom, je specifična varijanta grupe bolesti koje se karakterišu nekrotizirajućim vaskulitisom malih i srednjih krvnih sudova. Prema našem saznanju, udruženost Velsovog sindoma i eozinofilne granulomatoze sa poliangiitisom je retka, do sada opisana kod deset bolesnika. Prikaz bolesnika. Prikazana je bolesnica, stara 34 godine, sa trogodišnjom istorijom periodične pojave eritematoznih plakova na trupu i edematoznih plakova nalik celulitisu na donjim ekstremitetima. U ličnoj anamnezi navela je astmu u prethodnih godinu dana, rinosinuzitis i nazalnu polipozu. Biopsijom kože uočeno je prisustvo difuznog eozinofilnog infiltrata u dermu sa karakterističnim “plamenim figurama”. Daljim pretragama evidentirana je eozinofilija u perifernoj krvi (22,6%), obostrani maksilarni sinuzitis, eozinofilni infiltrati i mikroapcesi u bronhijalnom zidu i perikapilarni eozinofilni infiltrati u intersticijumu pluća, uočeni brohoskopijom, kao i transbronhijalnom biopsijom. Lečenje je započeto metilprednizolonom 0,5 mg/kg/dan uz postepeno snižavanje doze narednih 12 nedelja. Postignuta je kompletna remisija promena na koži, uz ponovnu pojavu u poslednje dve godine, što je zahtevalo ponavljanje terapije. Zaključak. Udruženost ova dva sindroma je neuobičajena, sa mogućom zajedničkom patogenetskom osnovom. Pretpostavljamo da Velsov sindrom može biti prethodni stadijum eozinofilne granulomatoze sa poli angiitisom i mišljenja smo da bolesnike treba ispitati u smislu postojanja eozinofilne granulomatoze sa poliangiitisom s obzirom na to da ove bolesti mogu imati klinička i laboratorijska preklapanja.
Reference
Wells GC, Smith NP. Eosinophilic cellulitis. Br J Dermatol 1979; 100(1): 101‒9.
Caputo R, Marzano AV, Vezzoli P, Lunardon L. Wells syn-drome in adults and children: a report of 19 cases. Arch Der-matol 2006; 142(9): 1157‒61.
Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, et al. Churg-Strauss syndrome. Autoimmun Rev 2015; 14(4): 341‒8.
Boura P, Sarantopoulos A, Lefaki I, Skendros P, Papadopoulos P. Eosinophilic cellulitis (Wells' syndrome) as a cutaneous reac-tion to the administration of adalimumab. Ann Rheum Dis 2006; 65(6): 839‒40.
Fujimoto N, Wakabayashi M, Kato T, Nishio C, Tanaka T. Wells syndrome associated with Churg-Strauss syndrome. Clin Exp Dermatol 2011; 36(1): 46‒8.
Huang CF, Chen YF, Wang WM, Chiang CP. Wells' syndrome associated with Churg-Strauss syndrome: correlation with mast cell distribution. Int J Dermatol 2013; 52(2): 214‒6.
Koh KJ, Warren L, Moore L, James C, Thompson GN. Wells' syn-drome following thiomersal-containing vaccinations. Australas J Dermatol 2003; 44(3): 199‒202.
Lee SC, Shin SS, Lee JB, Won YH. Wells syndrome associated with Churg-Strauss syndrome. J Am Acad Dermatol 2000; 43(3): 556‒7.
Lee SH, Roh MR, Jee H, Chung KY, Jung JY. Wells' syndrome associated with Churg-Strauss syndrome. Ann Dermatol 2011; 23(4): 497‒500.
Ozden MG, Yildiz L, Aydin F, Şenturk N, Canturk T, Turanli AY. Is it really possible to differentiate insect bite-like reac-tion and nodular variant of eosinophilic cellulitis in a healthy person? Eur J Dermatol 2009; 19(6): 635‒6.
Ratzinger G, Zankl J, Zelger B. Wells syndrome and its relation-ship to Churg-Strauss syndrome. Int J Dermatol 2013; 52(8): 949‒54.
Schuttelaar ML, Jonkman MF. Bullous eosinophilic cellulitis (Wells' syndrome) associated with Churg-Strauss syndrome. J Eur Acad Dermatol Venereol 2003; 17(1): 91‒3.
Fujii K, Tanabe H, Kanno Y, Konishi K, Ohgou N. Eosinophilic cellulitis as a cutaneous manifestation of idiopathic hypereo-sinophilic syndrome. J Am Acad Dermatol 2003; 49(6): 1174‒7.
Vaglio A, Casazza I, Grasselli C, Corradi D, Sinico RA, Buzio C. Churg-Strauss syndrome. Kidney Int 2009; 76(9): 1006‒11.
Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term fol-low-up of 96 patients. Medicine (Baltimore) 1999; 78(1): 26‒37.
Weins AB, Biedermann T, Weiss T, Weiss JM. Wells syndrome. J Dtsch Dermatol Ges 2016; 14(10): 989‒93.
Jakiela B, Szczeklik W, Plutecka H, Sokolowska B, Mastalerz L, Sanak M, et al. Increased production of IL-5 and dominant Th2-type response in airways of Churg-Strauss syndrome pa-tients. Rheumatol (Oxford) 2012; 51(10): 1887‒93.
Sable-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Int Med 2005; 143(9): 632‒8.
Sinico RA, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, et al. Prevalence and clinical significance of antineutrophil cy-toplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 2005; 52(9): 2926‒35.
Kallenberg CG. Churg-Strauss syndrome: just one disease enti-ty? Arthritis Rheum 2005; 52(9): 2589‒93.
Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990; 33(8): 1094‒100.
Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medi-cine (Baltimore) 1996; 75(1): 17‒28.
Räßler F, Lukács J, Elsner P. Treatment of eosinophilic celluli-tis (Wells syndrome) – a systematic review. J Eur Acad Der-matol Venereol 2016; 30(9): 1465‒79.
Oray M, Abu Samra K, Ebrahimiadib N, Meese H, Foster CS. Long-term side effects of glucocorticoids. Expert Opin Drug Saf 2016; 15(4): 457‒65.