vasculită urticariană asociată cu boala castleman, forma localizată

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<ul><li><p>221</p><p>* Clinica Dermatologic Scarlat Longhin, Bucureti.** Institutul de Boli Infecioase Matei Bal, Bucureti.*** Policlinica Cetatea Histria, Bucureti.</p><p>VASCULIT URTICARIAN ASOCIAT CU BOALACASTLEMAN, FORMA LOCALIZAT </p><p>PREZENTARE DE CAZ</p><p>URTICARIAL VASCULITIS ASSOCIATED WITHCASTLEMANS DISEASE, THE LOCALISED FORM </p><p>A CASE REPORT</p><p>ALINA ELENA ILIE*, V. BENEA*, SIMONA ROXANAGEORGESCU*, D. MUREAN*, ALICE RUSU*,MARIA GRIGORE*, I. MACAVEI*, ANTOANELA BONOIU*, ANCAMIHAELAMLIN*,</p><p>ELISABETAOTILIA BENEA**, LIANAMANOLACHE***, J.D. DIACONU*</p><p>Bucureti</p><p>CAZURI CLINICECLINICAL CASES</p><p>Summary</p><p>Castlemans disease is a very rare disordercharacterized by benign lymph-node hypertrophy. There aredescribed two forms: the localized form, in which only onelymph node is involved, often pauci-symptomatic, treatedby surgical excision with full recovery without relapse inalmost all cases, and the multicentric or systemic form withgeneral symptoms, polyadenopathy (neck, chest, mediastin,abdomen), organomegaly (liver, spleen) and, with a muchless favorable prognosis. The etiology remains unknown,but some recent studies have suggested that the humanherpesvirus 8 (HHV 8) can be involved, especially in themulticentric forms.</p><p>We present the case of a 60 year-old man who wasadmitted to our hospital for acute urticaria (with an aspectof vasculitic urticaria) and angioedema, started two weeksbefore presentation. At the physical examination weobserved a submandibular round tumor (8 cm in diameter),firm-elastic to palpation, painless, covered by normalepidermis, which occurred simultaneously with urticariaand increased with time. We have treated the urticaria with</p><p>Rezumat</p><p>Boala Castleman este o afeciune foarte rar,caracterizat prin hipertrofia benign a ganglionilorlimfatici. Exista dou forme clinice: forma localizat n careeste afectat un singur grup ganglionar, frecvent pauci-simptomatic, tratat prin excizie chirurgical radical,fr recidive n majoritatea cazurilor i formamulticentric, caracterizat prin manifestri sistemice,poliadenopatie (mediastin, regiunea cervical, abdomenetc.), organomegalie (ex: splin, ficat) i care are unprognostic mai puin favorabil dect forma localizat.Etiologia bolii ramne necunoscut; studii recente ausugerat implicarea herpesvirusului uman 8 (HHV-8), nspecial n cazul formelor multicentrice.</p><p>Prezentm cazul unui pacient n vrsta de 60 ani caresolicit o consultaie pentru o erupie urticarian acut (cuaspect de vasculit urticarian) nsoit de angioedem,aprut cu dou sptmni anterior internrii. La examenulfizic s-a decelat o formaiune tumoral sub-mandibular,rotund-ovalar, cu diametrul de aproximativ 8 cm, ferm-elastic, nedureroas, cu tegumentul supraiacent de aspectnormal, care a aprut odat cu urticaria i care a crescut</p><p>DermatoVenerol. (Buc.), 55: 221-234</p></li><li><p>222</p><p>DermatoVenerol. (Buc.), 55: 221-234</p><p>Prezentare de caz</p><p>Un pacient n vrst de 60 ani s-a prezentatpentru o erupie urticarian cu debut acut, avndcaracteristici clinice de vasculit urticarian(leziuni pruriginoase urticariene generalizate,figurate, nsoite de senzaie de arsur, carepersistau n aceeai localizare peste 48 ore, uneleleziuni avnd n centru aspect purpuric fig. 1) iangioedem (fig. 2), aprute n urm cu dousptmni. Pacientul nu urma nici un tratamentmedicamentos i nu avea istoric de alergiemedicamentoas.</p><p>Examenul clinic a evideniat, n afara erupieiurticariene, o formaiune tumoral subman-dibular rotund, cu diametrul de 8 cm, ferm-elastic la palpare, nedureroas, acoperit deepiderm de aspect normal, care a aprut simultancu erupia urticarian i care a crescut progresiv ndimensiuni (fig. 3). Investigaiile uzuale, cum ar fihemoleucograma i profilul biochimic (incluzndtestele funciei hepatice) au fost n limite normale,cu excepia creterii VSH; serologia HIV a fostnegativ. De asemenea, s-au evideniat cretereanivelului complexelor imune circulante ihipocomplementemie. Pacientul a fost tratatpentru vasculita urticarian cu antihistaminice ipentoxifilin, rspunsul fiind favorabil. Ulterior afost ndrumat ctre secia ORL unde tumora a fostexcizat complet. Examenul histopatologic altumorii a evideniat hiperplazie limfoidangiofolicular - forma hialino-vascular,sugernd diagnosticul de boal Castleman.Examenul computer tomograf nu a pus neviden alte adenopatii, astfel nct s-a stabilit</p><p>Case report</p><p>A 60 year-old man was referred to ourhospital for an urticarial eruption suggestingurticarial vasculitis (generalized pruriticurticarial lesions, accompanied by a burningsensation, that last for more than 48 hours in afixed location, with purpura within the lesions fig. 1) and angioedema (fig. 2), started two weeksago. The patient was on no regular medicationsand no drug allergy was mentioned.</p><p>Physical examination revealed a sub-mandibular round tumor (8 cm in diameter),firm-elastic to palpation, painless, covered bynormal epidermis, which occurred simulta-neously with urticaria and increased with time(fig. 3). Routine investigations such as full bloodcount and biochemical profile (including hepaticfunction tests) were found to be within normalrange, except an elevated ESR; HIV serology wasnegative. Hypocomplementaemia and circu-lating immune complexes were present. Thepatient was treated for the urticarial vasculitiswith antihistamines and pentoxifilin with a goodresponse. The patient was referred to the ENTdepartment where the tumor was completelyexcised. The histological examination of thelesion showed angiofollicular lymphoidhyperplasia the hyaline-vascular form,suggesting the Castlemans disease. Thecomputer tomography/CT examination revealedno widespread lymphadenopathy, so thediagnosis was Castlemans disease, the localizedform. The patient was periodically evaluated; one</p><p>progresiv n dimensiuni. Erupia urticarian a fost tratatsistemic cu antihistaminice si pentoxifilin, evoluia fiindfavorabil. Pentru formaiunea tumoral submandibularpacientul a fost ndrumat ctre serviciul ORL unde aceasta afost excizat complet. Examenul histopatologic al formaiuniia pus n eviden o hiperplazie limfoid angiofolicular,forma hialin-vascular, sugernd diagnosticul de boalCastleman. Examenul computer tomograf nu a pus ineviden alte adenopatii sau organomegalii, astfel nct s-astabilit diagnosticul de boal Castleman, forma localizat. Laun an de la intervenia chirurgical pacientul eraasimptomatic i fr recidiva tumorii.</p><p>Cuvinte cheie: vasculit urticarian, boalCastleman, HHV-8.</p><p>antihistamines and pentoxifilin with a good response andwe referred the patient to the Otolaryngology departmentwhere the tumor was completely excised. The histologicalexamination of the lesion showed angiofollicular lymphoidhyperplasia the hyaline-vascular form, suggesting theCastlemans disease. The computer tomography revealed nowidespread lymphadenopathy, so the diagnosis wasCastlemans disease, the localized form. We periodicallyevaluate the patient; one year after surgical excision norelapse was noticed.</p><p>Keywords: urticarial vasculitis, Castlemans disease,HHV-8.</p></li><li><p>diagnosticul de boal Castleman, forma localizat.Pacientul este sub supraveghere dermatologicperiodic; la un an de la excizia chirurgical, nu s-a observat recidiva tumorii (fig. 4).</p><p>Discuii</p><p>Boala Castleman este o afeciune foarte rar,cu evoluie de obicei benign, de etiologienecunoscut, caracterizat prin hipertrofiaganglionilor limfatici. De-a lungul timpului s-aufolosit numeroi termeni pentru a descrie aceastentitate limfoproliferativ, cum ar fi: hiperplazielimfoid angiofolicular, hiperplazie gigant aganglionilor limfatici, hamartom al ganglionilorlimfatici, hamartom limfoid angiomatos,limforeticulom folicular, limfom gigant benign,hiperplazie angiofolicular a ganglionilorlimfatici mediastinali etc.1,2</p><p>Boala a fost descris pentru prima dat n1954 i 1956 de ctre Castleman ca o mrire de</p><p>year after surgical excision no relapse wasnoticed (fig. 4).</p><p>Comment</p><p>Castlemans disease is a very rare, usuallybenign disorder of unknown etiology charac-terized by benign lymph-node hypertrophy.Many terms have been used over the years todescribe this lymphoproliferative disorder,including angiofollicular lymphoid hyperplasia,giant lymph node hyperplasia, lymph nodehamartoma, angiomatous lymphoid hamartoma,follicular lymphoreticuloma, benign giantlymphoma, angiofollicular mediastinal lymphnode hyperplasia.1,2</p><p>The entity was first described in 1954 and1956 by Castleman et al as a benign, localizedenlargement of hyperplastic lymph nodes.3,4 In1970 Flendrig reported that this disorder has twoseparate histologic features;5 based on these</p><p>223</p><p>DermatoVenerol. (Buc.), 55: 221-234</p><p>Fig. 1/Fig. 1 Fig. 2/Fig. 2</p><p>Fig. 3/Fig. 3 Fig. 4/Fig. 4</p></li><li><p>224</p><p>DermatoVenerol. (Buc.), 55: 221-234</p><p>volum benign, localizat, a ganglionilorlimfatici.3,4 n 1970, Flendrig a comunicat faptulc, din punct de vedere histopatologic, aceastafeciune are dou manifestri distincte;5 ulterior,n 1972, Keller a clasificat boala Castleman ndou variante histopatologice, hialino-vasculari, respectiv, plasmocito-celular.6</p><p>Din punct de vedere clinic, boala se poatemanifesta sub dou forme: localizat sau cuafectare multicentric; forma localizat este ceamai frecvent.</p><p>Aproximativ 90% din cazurile cu boalCastleman localizat sunt de tip hialino-vascular i apar de obicei la tineri. Este afectat unsingur grup ganglionar i de obicei pacienii suntpauci-simptomatici. Forma localizat poateafecta orice parte a corpului care prezint esutlimfatic, cele mai frecvente localizri fiindmediastinul (60%), regiunea cervical (14%),intra-abdominal (retroperitoneal, pancreas,pelvis etc. - 11%), i axilar (4%);7 de asemenea,s-au raportat cazuri de afectare la nivel faringian,parotidian, regiunea bucal, extremiti etc.8,9,10</p><p>Tratamentul este reprezentat de exciziachirurgical, care este urmat de recuperareatotal, fr recidive. n aproape toate cazurile,evoluia este benign.11</p><p>Majoritatea cazurilor formei multicentricesunt de tip plasmocito-celular, apar tipic ndecada a asea de via i au o evoluie maiagresiv. Forma multicentric a bolii Castlemanse caracterizeaz prin poliadenopatie i afectaremultiorganic. Pacienii au afectare sistemic carese manifest prin simptome constituionale (ex:fatigabilitate, febr, anorexie, scdere ponderal,transpiraii nocturne), adenopatii diseminate(regiunea cervical, mediastin, abdomen etc),organomegalie (hepato- i/sau splenomegalie),edeme, revrsat pleural i/sau pericardic, ascit,infecii recidivante, tulburri de cretere, afectareosoas, neuropatii. Pacienii pot dezvolta infiltratleptomeningeal i la nivelul sistemului nervoscentral, precum i miastenia gravis. Afectareasistemic poate fi suficient de sever pentru adetermina panci-topenie i afectare de organ (nspecial respiratorie i renal), precum i oc carenecesit internarea ntr-o unitate de terapieintensiv.</p><p>n unele cazuri, boala Castleman formamulticentric, se poate asocia cu sindromul</p><p>features, in 1972 Keller et al subclassifiedCastlemans disease into hyaline-vascular andplasma-cell variant.6</p><p>The disease can be present in two clinicalforms: as a solitary mass or as multicentricdisease; the localized form is more common.</p><p>Approximately 90% of cases of the localizedform are of the hyaline-vascular type. It is usuallypresent in young adults. Only one lymph node isinvolved and the patients are often pauci-symptomatic. Castlemans disease may originatein any part of the body that contains lymphoidtissue; the most commonly involved body partsare mediastinum (60%), neck (14%), intra-abdominal (retroperitoneum, pancreas, pelvis,etc. - 11%), and axilla (4%);7 the disease was alsobeen reported in the parapharyngeal, parotid orbuccal region, limbs etc.8,9,10 The localized form ofCastlemans disease is treated by surgicalexcision with full recovery without relapse; inalmost all cases typically follows a benigncourse.11</p><p>Most cases of the multicentric form are of theplasma-cell type, typically presents in the sixthdecade, and follows a more aggressive naturalhistory. Multicentric Castlemans disease ischaracterized by polylymphadenopathy andmultiorgan involvement. Patients present with asystemic illness that manifests as constitutionalsymptoms (e.g. fatigue, fever, anorexia, weightloss, and night sweats), disseminated lymphnodes (neck, chest, mediastin, abdomen, etc.),organomegaly (liver, spleen), oedema, pulmonaryand pericardial effusions, ascites, recurrentinfections, growth disturbance, bone destruction,neuropathy. Patients can develop leptomeningealand CNS infiltration, as well as myastheniagravis. The systemic symptoms can be severeenough to cause pancytopaenia and organ failure(particularly respiratory and renal), as well asshock requiring admission into an intensive careunit.</p><p>In some cases multicentric Castlemansdisease may be associated with POEMSsyndrome (Polyneuropathy, Organomegaly,Endocrine disorders, Monoclonal gammopathy/Mprotein, Skin disease; sin. Crow-Fukase disease).POEMS syndrome is associated with HHV-8infection and an increased level of serum IL-6,similar to Castlemans disease.12,13 Two thirds of</p></li><li><p>225</p><p>DermatoVenerol. (Buc.), 55: 221-234</p><p>POEMS (Polineuropatie, Organomegalie, anomaliiEndocrine, gamapatie Monoclonal, afectarecutanat; sinonim boala Crow-Fukase).Sindromul POEMS se poate asocia cu infecia cuHHV-8 i cu nivele serice crescute de IL-6, similarcu boala Castleman.12,13 Dou treimi dintrepacienii cu sindromul POEMS prezint formaplasmocito-celular. Testele de laborator potevidenia anemie, leucocitoz, trombocitoz,creterea VSH, hipergamaglobulinemie,hipoalbuminemie, modificri electroencefalo-grafice etc. Frecvent, creterea imunoglobulineloreste policlonal; uneori se poate detecta ogamapatie monoclonal, situaie n care boalaevolueaz spre sindromul POEMS; n astfel decazuri, 80% din lanurile uoare monoclonalesunt de tip lambda.</p><p>Forma multicentric a bolii Castleman areevoluie agresiv i frecvent conduce la decesprin complicaii infecioase sau neoplazii (ex:limfom, plasmocitom, sarcom Kaposi, sarcomfolicular cu celule dendritice). La pacienii cuforma multicentric a bolii Castleman s-araportat o asociere de 32% cu neoplazii.14,15</p><p>Frecvent, n absena infeciei HIV, formamulticentric a bolii Castleman se asociaz culimfom non-hodgkinian.16 75% dintre pacieniiHIV-pozitivi i 13% dintre cei HIV-negativi cuboal Castleman multicentric au sau vordezvolta sarcom Kaposi pe parcursul evoluieibolii.17 De asemenea, pacienii HIV-pozitivi cuboala Castleman multicentric au osusceptibilitate mai mare de a dezvolta compli-caii pulmonare.17,18 Evoluia clinic se poatecomplica i cu amiloidoz sistemic.</p><p>Boala Castleman pare a avea o predileciepentru brbai i apare mai frecvent ntre a douai a cincea decad de via. La pacienii HIV-pozitivi, boala apare la vrste mai tinere. La copii,incidena bolii este sczut;19 totui, cnd apare,evoluia este mai favorabil dect la aduli.</p><p>Boala Castleman este o afeciunelimfoproliferativ atipic, benign, caracterizatprin hiperplazie limfoid hipervascular cumecanism distinct i etiologie necunoscut; nu setie dac este un proces reactiv sau neoplazic.Printre posibilele cauze de apariie pot fihiperplazia focal secundar inflamaiei cronice,imunodeficiena, autoimunita...</p></li></ul>