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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 careconstã în prezenþa de glande sebacee ectopice pemucoasa buzelor, gingiilor, faþa internã a obrajilorºi care apar ca mici granule (milia) alb gãlbui. [1].

Prima descriere a fost fãcutã de mediculdermatolog american J.A.Fordyce în 1996. El adescris aceste granule alb gãlbui în zonavermicularã a buzelor, pe mucoasa oralã dar ºi pemucoasa genitalã [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 afecþiune foarte rãspânditã dar cuevoluþie lungã, asimptomaticã ce poate produce pacientuluidisconfort psihic ºi estetic. Simptomatologia clinicã esteuºor de recunoscut, iar diagnosticul diferenþial se face cupuþine afecþiuni, în principal cu adenoamele sebacee ºivegetaþiile veneriene în cazul localizãrii genitale.Mecanismul patogen ce duce la apariþia acestor glandesebacee ectopice nu este cunoscut dar este important demenþionat cã aceste glande sebacee nu sunt asociatefoliculului pilos. Evoluþia bolii este benignã iar tratamentulatunci când se impune este tratamentul ablativ.

Prezentãm cazul unui tânãr care a prezentat aceastãafecþiune din copilãrie ºi care a refuzat orice tratamentpreferând 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: Fordyce’s disease, clinical aspects,histopathological aspects.

Intrat în redacþie: 7.06.2014Acceptat: 7.08.2014

Received: 7.06.2014Accepted: 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 CLINICECLINICAL CASES

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În prezent se considerã cã boala Fordycereprezintã o anomalie legatã de glandele sebaceecare apar ectopic la nivelul mucoasei bucale saugenitale. La mucoasa genitalã au fost denumitegladele lui Tyson. Mai rar glandele sebaceeectopice se pot întâlni ºi la nivelul ochiului(glande meibomiene) sau la nivelul areoleimamare fiind denumite tuberculi Mongomeri [3].

Examinarea histologicã a granulelor Fordyceevidenþiazã faptul cã 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 înfundatsau absent necomunicând cu suprafaþaepiteliului.[5]. Localizarea acestor glande este înmare parte la nivelul dermului superficial iarproliferarea lor ar produce o ridicare circumscrisãa epidermului care este vizibilã pe suprafaþaacestuia sub forma unei mici granule gãlbuialbicioase.

Clinic, boala Fordyce se manifestã prinprezenþa unor mici papule de 1-2 mm cu ocoloraþie gãlbui albicioase, cu o consistenþãrelativ crescutã. Atunci când aceste mici papulesunt mai mari ºi sunt foarte dese (zeci ºi chiarsute) pot realiza aspectul unor mici placarde.

În cazul localizãrii bucale leziunile seîntâlnesc frecvent, în special pe mucoasa obrajilorîn cele mai multe cazuri bilateral, simetric, dar ºila nivelul regiunii vermiculare a buzeisuperioare. Leziunile se întâlnesc rar în regiuneaamigdalianã sau în zona faringianã [5].

La nivel genital granulele Fordyce prezintãacelaºi aspect de micã proeminenþã gãlbuialbicioasã cu diametru de 2-3 mm situate pelabiile mici sau penian în zona coroanei glan-dului. Leziunile sunt asimptomatice. Prezenþagranulelor Fordyce nu este asociatã cu alte bolidermatologice sau genetice [5]. În cazullocalizãrii genitale prezenþa granulelor Fordycenu este legatã de activitatea sexualã.

Evoluþia este benignã, leziunile apar încopilãrie, persistã sau pot sã aparã leziuni noiaproape toatã viaþa [6,7].

Caz clinic

Prezentãm cazul unui pacient de 26 ani carela examenul dermatologic a evidenþiat prezenþaunor papule de 1-3 mm, cu coloraþie gãlbui

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 Tyson’s 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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albicioasã, situate pe mucoasa feþei interne aobrazului drept pe o suprafaþã de aproximativ 4cm2 ºi la nivelul buzei superioare în special înzona vermicularã. Pe faþa internã a obrazuluistâng prezintã aceleaºi leziuni dar pe o suprafaþã

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 gãlbui albicioase pe mucoasa obrazului stâng

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

Fig. 2. Granule gãlbui albicioase pe mucoasa obrazuluidrept

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

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

Fig. 4. Lobul matur de glandã sebacee. O glandã cu ductînfundat. 4X, HE

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

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de 2-3 cm2. În zona genitalã nu s-au evidenþiatleziuni.

Leziunile au apãrut în jurul vârstei de 12 aniºi au progresat pânã la aspectul actual. Pacientulnu prezintã semne subiective iar prezentarea laexamenul dermatologic a fost fãcutã la indicaþiilemedicului stomatolog.

Se practicã la solicitarea pacientuluiexamenul histopatologic din o zonã cu papulegãlbui albicioase de pe faþa internã a obrazuluidrept. Rezultatul examenului histopatologic:epiteliu cu zone de acantozã ºi numeroase celuleveziculizate. În dermul superficial hiperplazie deglande sebacee, neasociate foliculului pilossituate în vecinãtatea joncþiunii epidermale.

Discuþii

Aºa cum este definitã, boala Fordyce constãîn prezenþa de glande sebacee ectopice care nusunt asociate firului de pãr.

Celulele glandulare sunt parþial funcþionaledar sebumul nu poate fi eliminat datoritãabsenþei sau înfundãrii canalului excretor.

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

Cauza apariþiei acestor glande ectopice nueste cunoscutã ºi nu s-au putut face asocieri întreapariþia acestor glande ºi afecþiuni virale,bacteriene sau alte tipuri de afecþiuni. În cazullocalizãrii genitale este clar cã nu existã o

the same lesions but in a smaller area, 2-3cm2.There were no lesions present in the genital area.

The lesions appeared around the age of 12and have progressed until the present day. Thepatient does not present any subjective signs andthe indication towards the dermatology examcame from his dentist.

Under the patient’s request, a histopatho-logical exam is conducted from an area withyellow-white coloring from the inner right cheek.

Result of the histopathological examination:epithelia with achantosis area and numerousvacuolised cells. In the superficial dermissebaceous glands hyperplasia non associatedwith hair follicle situated in the epidermaljunction area.

Discusions

As per its definition, the Fordyce disease isbased on the presence of ectopic sebaceousglands which are not associated with the hairstrand (follicle).

The glandular cells are partially functionalbut the sebum cannot be eliminated due to theabsence or blocking of the excretory canal.

There have been cases where the glands hadpresented an excretory canal that leads to theepithelial surface but does not associate to thehair follicle. There have been no notifications ofany change in the histological make-up of theglandular cells from the Fordyce granulescompared to the sebaceous gland cells associatedto the hair follicle.

The cause behind the apparition of theectopic glands is not known and an associationbetween these glands, viral affections, bacterial or

Fig. 5. Detaliu glandã sebacee cu duct înfundat. 10X, HEFig. 5. Detail, gland with blocked canal. 10X, HE

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transmitere pe cale sexualã. Clinic leziunile dinboala Fordyce trebuiesc deosebite de hiperplaziasebacee (papule gãlbui de obicei la nivelul feþei(tumorã sdolitarã, rarã), rar pe trunchi careprezintã o adânciturã care corespunde cuostiumul folicular, ca ºi de adenomul sebaceu(tumorã unicã rarã situatã la nivelul capului ºigâtului).

Frecvenþa mare de apariþie a acestor glandeectopice, în jur de 80% din populaþie, când seconsiderã ºi prezenþa câtorva granule, a fãcut sãse considere cã aceste glande ar fi, fie varianteanatomice, fie adenoame sebacee. În prezent seconsiderã cã aceste glande sunt glande ectopice,care nu sunt asociate foliculului pilos [8].

Glandele ectopice din boala Fordyce, chiardacã sunt parþial funcþionale ºi chiar dacã încazuri rare prezintã un canal înfundat care sepoate deschide la suprafaþa epiteliului sedeosebesc de glandele sebacee din acneeavulgarã, unde aceste glande sunt asociate unuifolicul pilos iar astuparea canalului excrertor seface în urma unor procese patologice.

Boala Fordyce trebuie delimitatã de boalaFox-Fordyce. Chiar dacã aspectul clinic ºihistopatologic este diferit, asemãnarea de numepoate crea confuzii. În boala Fox-Fordyce existã oînfundare a canalului secretor al glandei apocrinecu un dop de keratinã. Clinic apar mici papuleperifoliculare uºor pigmentare situate în axilã, înzona anogenitalã ºi perianalã. Uneori leziunilesunt pruriginoase [9].

Evoluþia bolii Fordyce este benignã, pacienþiineavând niciun simptom.

În cele mai multe cazuri nu este nevoie detratament. Se poate face, din considerenteestetice, cel mai frecvent se utilizeazã excizia prinintermediul laserului CO2 sau aplicarea de geluriorale cu tretionin sau acid tricloracetic [4,10].

Concluzii

În cazul pacientului prezentat, acesta avealeziuni cu extindere moderatã, fãrã leziunigenitale în momentul examinãrii, fãrã acuzesubiective, cu extindere lentã.

Pacientul a refuzat orice tip de tratament ºi apreferat consilierea sub forma unei discuþiidespre aceastã afecþiune.

other types of affections could not be made. In thecase of the genital localization it is clear that thereis not a sexual transmission involved.

Clinically Fordyce diseases lesions must bedistinguished by sebaceous hyperplasia (yellowpapules usually at the face level, rarely on trunk)that presents a gape corresponding with follicleostium , as by the sebaceous adenoma (uniquerare tumour situated on head or neck).

The high occurring frequency of these ectopicglands, around 80% of the population, when evena few granules are taken into account, has led tobelief that these glands could be eitheranatomical variations or sebaceous adenomas. Inpresent day it is considered that these glands areectopic glands, which are not associated to thehair follicle [8].

The ectopic glands in Fordyce disease, even ifpartly functional and even if in some rare casesthey present a blocked canal which may open tothe surface of the epithelia it distinguishes fromthe sebaceous glands in vulgar acne, where theseglands are associated to a hair follicle and theblocking the of excretory canal takes place due tocertain pathological processes.

The Fordyce disease must be delimitatedfrom the Fox-Fordyce disease. Even if the clinicaland histopathological aspect is different, thename resemblance may lead to confusion. In theFox-Fordyce disease there is a clogging of thesecretory canal of the apocrine gland by means ofa keratin plug. From a clinical viewpoint, smallperifollicular papules appear, they are slightlypigmented in the armpit region, also in theanogenital and perianal area. Sometimes thelesions present itching [9].

The evolution of Fordyce disease is benign,patients not having any symptoms.

In most cases, treatment is not needed. It canprovided for aesthetic reasons, and mostfrequently excision is utilized using a CO2 laseror the application of oral gels containing tretioninor tricloracetic acid [4,10].

ConclusionsIn the case of the presented patient, there are

lesions with moderate expansion present,without genital lesions at the moment of theexamination, with no subjective accusations, witha slow expansion. The patient refused any sort oftreatment and preferred counseling under theform of discussion regarding this affection.

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Ann.Dermatol. 2012, 24, (1), 103-106.7. Gorsky M., Buchner A., Fundoianu-Dayan D., Cohen C. Fordyce’s granule in the oral mucosa of adult Israeli Jews

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Buccale, 1981, 9(2), 109-128.9. Miller J.L., Hurtley H.J. Disease of eccrine and apocrine sweat glands in Dermatology. Second Ed. Editors

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CO2/laser In Dermatology Surgery, 2003, 29, (8), 867-871.

Conflict de interese Conflict of interestNEDECLARATE NONE DECLARED

Adresa de corespondenþã: alecu.mihail@gmail.com

Correspondance address: alecu.mihail@gmail.com