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157 BOALA FORDYCE. CAZ CLINIC FORDYCE DISEASE. CLINICAL CASE V. NEAMÞU * , GABRIELA COMAN * , MIOARA GRIGORE * , IRINA DIMA * , M.G. RUSSU * , M. ALECU*,** O definiþie simplã considerã boala Fordyce (Fordyce granule, Fordyce spots) o afecþiune care constã în prezenþa de glande sebacee ectopice pe mucoasa buzelor, gingiilor, faþa internã a obrajilor ºi care apar ca mici granule (milia) alb gãlbui. [1]. Prima descriere a fost fãcutã de medicul dermatolog american J.A.Fordyce în 1996. El a descris aceste granule alb gãlbui în zona vermicularã a buzelor, pe mucoasa oralã dar ºi pe mucoasa genitalã [2]. A simple definition considers the Fordyce disease (Fordyce granules, Fordyce spots) an affection which is based on the presence of ectopic sebaceous glands on the mucosa of the lips and gums, the inside of the cheeks, which appear as small granules (milia) in a white to yellowish shade [1]. The first description was made by American dermatologist J.A. Fordyce in 1996. He described these white-yellowish granules in the vermicular Rezumat Boala Fordyce este o afecþiune foarte rãspânditã dar cu evoluþie lungã, asimptomaticã ce poate produce pacientului disconfort psihic ºi estetic. Simptomatologia clinicã este uºor de recunoscut, iar diagnosticul diferenþial se face cu puþine afecþiuni, în principal cu adenoamele sebacee ºi vegetaþiile veneriene în cazul localizãrii genitale. Mecanismul patogen ce duce la apariþia acestor glande sebacee ectopice nu este cunoscut dar este important de menþionat cã aceste glande sebacee nu sunt asociate foliculului pilos. Evoluþia bolii este benignã iar tratamentul atunci când se impune este tratamentul ablativ. Prezentãm cazul unui tânãr care a prezentat aceastã afecþiune din copilãrie ºi care a refuzat orice tratament preferând consilierea. Cuvinte cheie: boala Fordyce, aspecte clinice, aspecte histopatologice. Summary Fordyce Disease is a very common condition but with long asymptomatic evolution that can cause psychological and aesthetic discomfort. Clinical symptomatology is easily recognizable, and the differential diagnosis is made with few ailments, mainly with sebaceous adenomas and genital warts in the case of genital localisation. Pathogenic mechanism leading to the occurrence of these ectopic sebaceous glands is not known but it is important to note that these sebaceous glands are not associated with hair follicles. The evolution of the disease is benign and the treatment, when is required is surgical ablation. We present the case of a young man who pesented this condition since childhood and who refused any treatment preferring counseling. Keywords: Fordyce’s disease, clinical aspects, histopathological aspects. Intrat în redacþie: 7.06.2014 Acceptat: 7.08.2014 Received: 7.06.2014 Accepted: 7.08.2014 * Spitalul Clinic de Boli Infecþioase ºi Tropicale Dr. Victor Babeº, Bucureºti. Clinical Hospital for Infectious and Tropical Diseases Dr. Victor Babes, Bucharest. ** Universitatea Titu Maiorescu, Facultatea de Medicinã, Bucureºti. Titu Maiorescu University, Faculty of Medicine, Bucharest. CAZURI CLINICE CLINICAL CASES

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  • 157

    BOALA FORDYCE. CAZ CLINIC

    FORDYCE DISEASE. CLINICAL CASE

    V. NEAMU*, GABRIELA COMAN*, MIOARA GRIGORE*, IRINA DIMA*, M.G. RUSSU*, M. ALECU*,**

    O definiie simpl consider boala Fordyce(Fordyce granule, Fordyce spots) o afeciune careconst n prezena de glande sebacee ectopice pemucoasa buzelor, gingiilor, faa intern a obrajilori care apar ca mici granule (milia) alb glbui. [1].

    Prima descriere a fost fcut de mediculdermatolog american J.A.Fordyce n 1996. El adescris aceste granule alb glbui n zonavermicular a buzelor, pe mucoasa oral dar i pemucoasa genital [2].

    A simple definition considers the Fordycedisease (Fordyce granules, Fordyce spots) anaffection which is based on the presence ofectopic sebaceous glands on the mucosa of thelips and gums, the inside of the cheeks, whichappear as small granules (milia) in a white toyellowish shade [1].

    The first description was made by Americandermatologist J.A. Fordyce in 1996. He describedthese white-yellowish granules in the vermicular

    Rezumat

    Boala Fordyce este o afeciune foarte rspndit dar cuevoluie lung, asimptomatic ce poate produce pacientuluidisconfort psihic i estetic. Simptomatologia clinic esteuor de recunoscut, iar diagnosticul diferenial se face cupuine afeciuni, n principal cu adenoamele sebacee ivegetaiile veneriene n cazul localizrii genitale.Mecanismul patogen ce duce la apariia acestor glandesebacee ectopice nu este cunoscut dar este important demenionat c aceste glande sebacee nu sunt asociatefoliculului pilos. Evoluia bolii este benign iar tratamentulatunci cnd se impune este tratamentul ablativ.

    Prezentm cazul unui tnr care a prezentat aceastafeciune din copilrie i care a refuzat orice tratamentprefernd consilierea.

    Cuvinte cheie: boala Fordyce, aspecte clinice, aspectehistopatologice.

    Summary

    Fordyce Disease is a very common condition but withlong asymptomatic evolution that can cause psychologicaland aesthetic discomfort. Clinical symptomatology is easilyrecognizable, and the differential diagnosis is made withfew ailments, mainly with sebaceous adenomas and genitalwarts in the case of genital localisation. Pathogenicmechanism leading to the occurrence of these ectopicsebaceous glands is not known but it is important to notethat these sebaceous glands are not associated with hairfollicles. The evolution of the disease is benign and thetreatment, when is required is surgical ablation.

    We present the case of a young man who pesented thiscondition since childhood and who refused any treatmentpreferring counseling.

    Keywords: Fordyces disease, clinical aspects,histopathological aspects.

    Intrat n redacie: 7.06.2014Acceptat: 7.08.2014

    Received: 7.06.2014Accepted: 7.08.2014

    * Spitalul Clinic de Boli Infecioase i Tropicale Dr. Victor Babe, Bucureti.Clinical Hospital for Infectious and Tropical Diseases Dr. Victor Babes, Bucharest.

    ** Universitatea Titu Maiorescu, Facultatea de Medicin, Bucureti.Titu Maiorescu University, Faculty of Medicine, Bucharest.

    CAZURI CLINICECLINICAL CASES

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    n prezent se consider c boala Fordycereprezint o anomalie legat de glandele sebaceecare apar ectopic la nivelul mucoasei bucale saugenitale. La mucoasa genital au fost denumitegladele lui Tyson. Mai rar glandele sebaceeectopice se pot ntlni i la nivelul ochiului(glande meibomiene) sau la nivelul areoleimamare fiind denumite tuberculi Mongomeri [3].

    Examinarea histologic a granulelor Fordyceevideniaz faptul c aceste glande sunt formatedin glande sebacee similare cu glandele sebaceedin derm, sunt uni sau pluri lobate, dar acesteglande nu sunt asociate foliculului pilos (glandesebacee libere) iar canalul excretor este nfundatsau absent necomunicnd cu suprafaaepiteliului.[5]. Localizarea acestor glande este nmare parte la nivelul dermului superficial iarproliferarea lor ar produce o ridicare circumscrisa epidermului care este vizibil pe suprafaaacestuia sub forma unei mici granule glbuialbicioase.

    Clinic, boala Fordyce se manifest prinprezena unor mici papule de 1-2 mm cu ocoloraie glbui albicioase, cu o consistenrelativ crescut. Atunci cnd aceste mici papulesunt mai mari i sunt foarte dese (zeci i chiarsute) pot realiza aspectul unor mici placarde.

    n cazul localizrii bucale leziunile sentlnesc frecvent, n special pe mucoasa obrajilorn cele mai multe cazuri bilateral, simetric, dar ila nivelul regiunii vermiculare a buzeisuperioare. Leziunile se ntlnesc rar n regiuneaamigdalian sau n zona faringian [5].

    La nivel genital granulele Fordyce prezintacelai aspect de mic proeminen glbuialbicioas cu diametru de 2-3 mm situate pelabiile mici sau penian n zona coroanei glan-dului. Leziunile sunt asimptomatice. Prezenagranulelor Fordyce nu este asociat cu alte bolidermatologice sau genetice [5]. n cazullocalizrii genitale prezena granulelor Fordycenu este legat de activitatea sexual.

    Evoluia este benign, leziunile apar ncopilrie, persist sau pot s apar leziuni noiaproape toat viaa [6,7].

    Caz clinic

    Prezentm cazul unui pacient de 26 ani carela examenul dermatologic a evideniat prezenaunor papule de 1-3 mm, cu coloraie glbui

    area of the lips, on the oral mucosa as well on thegenital mucosa [2].

    Today it is considered that Fordyce diseaserepresents an anomaly linked to the sebaceousglands which appear in an ectopic fashion at theoral or genital mucosa level. In the case of thegenital mucosa appearance they have beennamed Tysons glands. In more rare situations, theectopic sebaceous glands can be found at eye level(meibomien glands) or in the mammary areolaarea by the name of Montgomery tubercles [3].

    The histological examination of the Fordycegranules outlines that these glands are formedfrom sebaceous glands, similar to the sebaceousglands in the derma, either singular or multilobed, however these are not associated with thehair follicle (free sebaceous glands) and theexcreting canal is either blocked or absent notcommunicating with the surface of the epithelia[5]. The location of these glands is mostly on thesuperficial derma level and their proliferationproduces a certain elevation of the epidermallayer which is visible on its surface as a smallwhite-yellowish granule.

    Clinically the Fordyce disease presents somesmall papules around 1-2 mm in size with ayellow-white color, with a rather increasedconsistency. When these small papules are larger,in both size and number, (up to tens or evenhundreds) can achieve the aspect of small placards.

    In the case of the oral located lesions, theseare found frequently, especially on the cheeksmucosa in most cases present on both sides,symmetrically, also in the vermicular region ofthe upper lip. The lesions are rarely present in theamygdala region or the pharyngeal area [5].

    In the genital area the Fordyce granulespresent the same small prominence white-yellowish visual aspect with a 2-3 mm sizelocated on the smaller labia or at a penial level inthe crown part of the gland. The lesions areasymptomatic. The presence of the Fordycegranules is not associated with otherdermatological or genetic diseases [5]. In thegenital localization the presence of the Fordycegranules is not connected to sexual activity.

    Evolution is benign, lesions showing up inchildhood, which may persist or have new onesappear over time [6,7].

    Clinical CaseWe present the case of a 26 year old patient

    who in the course of a dermatology examination

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    albicioas, situate pe mucoasa feei interne aobrazului drept pe o suprafa de aproximativ 4cm2 i la nivelul buzei superioare n special nzona vermicular. Pe faa intern a obrazuluistng prezint aceleai leziuni dar pe o suprafa

    pointed out to the presence of some papules 1-3mm in size, showing a yellow to white color,located on the inner right cheek mucosa on a 4cm2

    area, well as the upper lip, especially in thevermicular area. On the inner left cheek he presents

    Fig. 1. Granule glbui albicioase pe mucoasa obrazului stng

    Fig. 1. White to yellowish small granules on the left cheek mucosa

    Fig. 2. Granule glbui albicioase pe mucoasa obrazuluidrept

    Fig. 2. White to yellowish on the right cheek mucosa

    Fig. 3. Granule alb glbui pe mucoasa buzei superioareFig. 3. White to yellowish on the superior lip mucosa

    Fig. 4. Lobul matur de gland sebacee. O gland cu ductnfundat. 4X, HE

    Fig. 4. Sebaceous gland mature lobe. Gland with blockedsecretory canal. 4X, HE

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    de 2-3 cm2. n zona genital nu s-au evideniatleziuni.

    Leziunile au aprut n jurul vrstei de 12 anii au progresat pn la aspectul actual. Pacientulnu prezint semne subiective iar prezentarea laexamenul dermatologic a fost fcut la indicaiilemedicului stomatolog.

    Se practic la solicitarea pacientuluiexamenul histopatologic din o zon cu papuleglbui albicioase de pe faa intern a obrazuluidrept. Rezultatul examenului histopatologic:epiteliu cu zone de acantoz i numeroase celuleveziculizate. n dermul superficial hiperplazie deglande sebacee, neasociate foliculului pilossituate n vecintatea jonciunii epidermale.

    Discuii

    Aa cum este definit, boala Fordyce constn prezena de glande sebacee ectopice care nusunt asociate firului de pr.

    Celulele glandulare sunt parial funcionaledar sebumul nu poate fi eliminat datoritabsenei sau nfundrii canalului excretor.

    S-au evideniat cazuri cnd glandele auprezentat canal excretor care ajunge la suprafaaepiteliului dar nu este asociat foliculului pilos.Nu s-au semnalat modificri histologice alecelulelor glandulare din granulele Fordycecomparativ cu celulele glandelor sebacee asociatefoliculului pilos.

    Cauza apariiei acestor glande ectopice nueste cunoscut i nu s-au putu