pseudopelada brocq pseudopelade of brocq...plãci diseminate pe suprafaþa scalpului, plãci mari...

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79 PSEUDOPELADA BROCQ PSEUDOPELADE OF BROCQ ALEXANDRU OANÞÃ* Summary Pseudopelade of Brocq is a chronic, slowly progressive, lymphocytic cicatricial alopecia whose exact etiology remains unknown. Described by Brocq in 1905 pseudopelade is characterized by asymptomatic, porcelain white or ivory, round or oval, atrophic plaques, with smooth surface without hair follicles, usually noninflammatory, localized on the vertex and parietal areas of the scalp giving the appearance of „en empreinte de pas dans la neige”. Dermoscopy confirm the absence of hair follicles and perifollicular inflammation and hyperkeratosis. Histopathology is usually nonspecific, in early-stage showing a perifollicular and perivascular lymphocytic infiltrate, and in the late-stage a dermal fibrosis with destruction of the hair follicle. The differential diagnosis is made with alopecia areata and cicatricial alopecia: discoid lupus erythematous, lichen planopilaris, central centrifugal cicatricial alopecia, etc. The treatment is mainly made with topical corticosteroids, intralesional triamcinolone acetonide and in the acute stages of the disease systemic corticosteroids, cyclosporine and mycophenolate mofetil are required. Key words: pseudopelade, pelada, cicatricial alopecia, lichen planopilaris, discoid lupus erythematous. * S.C. Dermamed S.R.L. Braºov, România. Dermamed Ltd. Braºov, Romania. Rezumat Pseudopelada Brocq este o alopecie cicatricialã limfocitarã cronicã cu evoluþie lent progresivã a cãrei etiopatogenie exactã rãmâne necunoscutã. Descrisã de Brocq în 1905 se caracterizeazã prin plãci alopecice, atrofice, netede, cu absenþa foliculilor piloºi, rotunde sau ovale, de obicei neinflamatorii, de culoare albã sidefie, asimptomatice, localizate la nivelul vertexului ºi zonelor parietale ale scalpului dând aspectul de „en empreinte de pas dans la neige”. Dermatoscopia confirmã absenþa foliculilor piloºi ºi a inflamaþiei perifoliculare ºi hiperkeratozei. Histopatologia este nespecificã, iniþial fiind prezent un infiltrat limfocitar peripilar ºi perivascular pentru ca în stadiul tardiv sã aparã o fibrozã dermicã cu distrugerea foliculului pilos. Diagnosticul diferenþial se face cu pelada ºi cu alopeciile cicatriciale: lupusul eritematos cronic, lichenul plan pilar, alopecia cicatricialã centrifugã, etc. Tratamentul se efectueazã în principal cu dermatocorticoizi, triamcinolon acetonid intralezional, iar corticoterapia generalã, ciclosporina ºi micofenolatul mofetil se impun în fazele acute ale bolii. Cuvinte cheie: pseudopeladã, pelada, alopecie cicatricialã, lichen planopilar, lupus eritematos discoid Intrat în redacþie: 15.04.2015 Acceptat: 26.05.2015 Received: 15.04.2015 Accepted: 26.05.2015 CAZURI CLINICE CLINICAL CASES

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Page 1: PSEUDOPELADA BROCQ PSEUDOPELADE OF BROCQ...plãci diseminate pe suprafaþa scalpului, plãci mari sau combinarea celor douã forme clinice. Iniþial plãcile sunt de dimensiuni mici,

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PSEUDOPELADA BROCQ

PSEUDOPELADE OF BROCQ

ALEXANDRU OANÞÃ*

Summary

Pseudopelade of Brocq is a chronic, slowly progressive,lymphocytic cicatricial alopecia whose exact etiologyremains unknown. Described by Brocq in 1905pseudopelade is characterized by asymptomatic, porcelainwhite or ivory, round or oval, atrophic plaques, with smoothsurface without hair follicles, usually noninflammatory,localized on the vertex and parietal areas of the scalp givingthe appearance of „en empreinte de pas dans la neige”.Dermoscopy confirm the absence of hair follicles andperifollicular inflammation and hyperkeratosis.Histopathology is usually nonspecific, in early-stageshowing a perifollicular and perivascular lymphocyticinfiltrate, and in the late-stage a dermal fibrosis withdestruction of the hair follicle. The differential diagnosis ismade with alopecia areata and cicatricial alopecia: discoidlupus erythematous, lichen planopilaris, central centrifugalcicatricial alopecia, etc. The treatment is mainly made withtopical corticosteroids, intralesional triamcinoloneacetonide and in the acute stages of the disease systemiccorticosteroids, cyclosporine and mycophenolate mofetil arerequired.

Key words: pseudopelade, pelada, cicatricial alopecia,lichen planopilaris, discoid lupus erythematous.

* S.C. Dermamed S.R.L. Braºov, România.

Dermamed Ltd. Braºov, Romania.

Rezumat

Pseudopelada Brocq este o alopecie cicatricialãlimfocitarã cronicã cu evoluþie lent progresivã a cãreietiopatogenie exactã rãmâne necunoscutã. Descrisã deBrocq în 1905 se caracterizeazã prin plãci alopecice,atrofice, netede, cu absenþa foliculilor piloºi, rotunde sauovale, de obicei neinflamatorii, de culoare albã sidefie,asimptomatice, localizate la nivelul vertexului ºi zonelorparietale ale scalpului dând aspectul de „en empreinte depas dans la neige”. Dermatoscopia confirmã absenþafoliculilor piloºi ºi a inflamaþiei perifoliculare ºihiperkeratozei. Histopatologia este nespecificã, iniþial fiindprezent un infiltrat limfocitar peripilar ºi perivascularpentru ca în stadiul tardiv sã aparã o fibrozã dermicã cudistrugerea foliculului pilos. Diagnosticul diferenþial seface cu pelada ºi cu alopeciile cicatriciale: lupusuleritematos cronic, lichenul plan pilar, alopecia cicatricialãcentrifugã, etc. Tratamentul se efectueazã în principal cudermatocorticoizi, triamcinolon acetonid intralezional, iarcorticoterapia generalã, ciclosporina ºi micofenolatulmofetil se impun în fazele acute ale bolii.

Cuvinte cheie: pseudopeladã, pelada, alopeciecicatricialã, lichen planopilar, lupus eritematos discoid

Intrat în redacþie: 15.04.2015Acceptat: 26.05.2015

Received: 15.04.2015Accepted: 26.05.2015

CAZURI CLINICECLINICAL CASES

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Pseudopelada Brocq (PPB) este o alopeciecicatricialã limfocitarã cronicã cu evoluþie lentprogresivã a cãrei etiopatogenie exactã rãmâne încontinuare necunoscutã [1, 2]. PPB reprezintãîntre 10% ºi 40% din ansamblul alopeciilorcicatriciale limfocitare [3, 4]. Alopecia cicatricialãprimarã a fost denumitã „pseudopeladã” deBrocq datoritã asemãnãrii cu pelada. În 1905Brocq, Lenglet ºi Ayrignac [5], observând la 29 depacienþi caracterele clinice ale PPB, trag concluziacã pseudopelada este o entitate nosologicãdistinctã. Aceastã pãrere nu este însã acceptatã înunanimitate, existând autori care considerã PPB oformã evolutivã a altor alopecii cicatricialeprimare precum lichenul plan pilar (LPP) saulupusul eritematos cronic (LEC) [6, 7] sau stadiulfinal al mai multor alopecii cicatriciale (étatpseudopeladique) [8]. Existã ºi autori care considerãtermenul de pseudopeladã depãºit, în opinia loracesta trebuind sã fie abandonat [9, 10, 11].

Brocq ºi alþi autori considerã ca ºicaracteristici clinice ale PPB prezenþa plãciloratrofice, ovale sau rotunde, de coloraþie alb-sidefie, fãrã prezenþa semnelor inflamatorii. Înschimb, Braun-Falco ºi colab. [12], observând 26de pacienþi diagnosticaþi cu PPB, gãsesc prezenþaeritemului în cursul evoluþiei bolii la majoritateaacestora (86%), iar la examenul histologic suntprezente aspecte de LEC ºi LPP la 33% dintreaceºtia. La fel ºi Amato ºi colab., [13] gãsesc la 36de pacienþi diagnosticaþi cu PPB la examenulhistologic în stadiile timpurii ale afecþiunii aspectde LEC ºi LPP la 69% dintre aceºtia. NorthAmerican Hair Research Society defineºte PPBprin prezenþa plãcilor alopecice discrete, netede,de coloraþia cãrnii (flesh-toned), fãrã prezenþahiperkeratozei foliculare sau inflamaþieiperifoliculare [14]. Se remarcã lipsa menþionãriiatrofiei dar menþionarea coloraþiei cãrnii pentruincluderea pacienþilor negri la care aspectul clasicalb-sidefiu lipseºte.

În patogenia PPB sunt incriminaþi o serie defactori precum natura autoimunã a afecþiunii,infecþia cu Borrelia [15] sau îmbãtrânireaprematurã a rezervorului de celule stemfoliculare.

PPB este caracterizatã clinic prin prezenþa deplãci alopecice de dimensiuni mici, rotunde sauovale, de obicei neinflamatorii, de culoare albãsidefie sau de culoarea cãrnii. Plãcile sunt

Pseudopelade of Brocq (PPB) is a chroniclymphocytic cicatricial alopecia with a slowlyprogressive evolution, whose exact etiologyremains unknown [1, 2]. PPB represents between10% and 40% of all lymphocytic cicatricialalopecia [3, 4]. Primary cicatricial alopecia wasnamed „pseudo-pelade“ by Brocq because of theresembling with pelada. In 1905 Brocq, Lengletand Ayrignac [5], noticing the clinicalcharacteristics of 29 patients with PDB, concludethat the pseudopelade is a distinct nosologicalentity. However, this opinion is not unanimouslyaccepted, some authors considering it to be anevolutionary form of other primary cicatricialalopecia like lichen planopilaris (LPP), discoidlupus erythematosus (DLE) [6, 7] or the end-stageof several cicatricial alopecia (état pseudo-peladique) [8]. There are authors who considerthe term „pseudopelade“ exceeded and in theiropinion it should be abandoned [9, 10, 11].

Brocq and other authors consider the clinicalcharacteristics of the PPB to be the presence ofoval or round, porcelain white, atrophic plaques,without any inflammatory signs. On the contrary,Braun-Falco et al. [12], observing 26 patientsdiagnosed with PPB, find that the majority of thepatients (86%) presented erythema during thedisease’s progression and histologically wherefound aspects of DLE and LPP in 33% of patients.Also Amato et al. [13] found in 36 patientshistological diagnosed with PPB in the earlystages histological appearance of DLE and LPP(69% of patients). North American Hair ResearchSociety defines PDB by the presence of smooth,discrete, flesh-toned alopecia plaques, withoutthe presence of follicular hyperkeratosis orperifollicular inflammation [14]. It is noted thatthe atrophy is not mentioned, but there is thementioning of flesh-toned coloration forincluding the african-american patients in whichthe pearly-white classic aspect is missing.

A number of factors are incriminated in PPB’spathogenesis such as the autoimmune nature ofthe disease, Borrelia infection [15], or prematureaging of reservoir follicle stem cells.

PPB is clinically characterized by thepresence of small round or oval, alopecic plaques,usually non-inflammatory, of porcelain-white orflesh-toned color. The plaques are atrophic,smooth, with no hair follicles, usually

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atrofice, netede, cu absenþa foliculilor piloºi, deobicei asimptomatice (fig. 1). Uneori în momentulapariþiei, plãcile pot fi roze, uneori discretedematoase sau chiar scuamoase. Vertexul ºizonele parietale ale scalpului sunt de obiceiafectate, observându-se însã ºi alte localizãri.Brocq a descris trei aspecte clinice ale PPB: miciplãci diseminate pe suprafaþa scalpului, plãcimari sau combinarea celor douã forme clinice.Iniþial plãcile sunt de dimensiuni mici, mãsurândîntre 5 ºi 10 mm, fiind similare unui bob de linte(une petite lentille) grupate pe aceeaºi regiune ºiavând aspectul amprentei paºilor pe zãpadã (enempreinte des pas dans la neige) sau amprenteiextremitãþii degetelor în fãinã (en empreintes del’extrémité des doigts dans la farine).

În evoluþie pot apãrea numeroase plãci micidând aspectul de confetti, posibilitatea extinderiireticulate a leziunilor lenticulare sau coalescenþaplãcilor mici în placarde mari alopecice cumargini neregulate sau policiclice cu dimensiunide câþiva centimetri, uneori înconjurate de plãcimici satelite. În faza activã a bolii firele de pãrsituate în periferia plãcilor pot fi smulse uºor(pull test pozitiv). Se pot observa fire de pãrgrupate sau izolate pe suprafaþa placardeloralopecice, deseori urmarea fuziunii a douã plãci.Au fost publicate ºi douã cazuri de PPB a bãrbii[4].

Dermatoscopia confirmã absenþa foliculilorpiloºi în centrul plãcii ºi a inflamaþiei perifoli-culare ºi hiperkeratozei foliculare caracteristiceLPP.

Clinic evoluþia bolii este insidioasã ºi lentprogresivã în decursul anilor cu apariþia de plãcinoi ºi ulterior confluarea acestora. Pacienþiidescoperã alopecia întâmplãtor, aceºtia negãsindpãr pe piaptãn sau perie cum remarca Degos [3].Alopecia este definitivã, procesul evolutivoprindu-se spontan dupã câþiva ani [1, 3].Evoluþia rapidã este neobiºnuitã [5, 16].

Histopatologic modificãrile prezente în acestcontext susþin cã PPB rãmâne un diagnostic deexcludere. În leziunile recente apare un infiltratlimfocitar moderat peripilar ºi perivascular, zonade interfaþã nefiind afectatã. Ulterior apare atrofiaepidermului infundibular ºi hiperplazia lamelarãconcentricã „în foi de ceapã” (en oignon) în jurulporþiunii superioare a foliculului pilos. Glandelesebacee sunt iniþial reduse în dimensiuni cu

asymptomatic (fig. 1). Sometimes, when theyappear, the plaques may be pink, sometimesdiscreetly edematous or even squamous. Thevertex and the parietal areas of the scalp areusually affected, other locations are alsoobserved. Brocq described three clinical aspectsof PPB: small plaques disseminated on the scalp,large plaques or combining the two clinicalforms. Initially the plaques are small, measuringbetween 5 and 10 mm, similar to a grain of lentil(une petite lentille) grouped in the same area andhaving the appearance of footprint in snow (enempreinte des pas dans la neige) or fingerprint onflour (en empreintes de l’extrémité des doigts dans lafarine).

Numerous small plaques may develop inevolution of the condition resulting in a confetti-like distribution, reticular extension of lenticularlesions or coalescence of small plaques into largeplaques with irregular or polycyclic borders, offew centimeters in size, sometimes surroundedby small satellite plaques. In the active phase ofthe disease, hairs located in the peripheral area ofthe plaque can be easily pulled out (positive pulltest). Grouped or isolated hairs can be seen on thesurface of the alopecic plaques, often followingthe merger of two plaques. There were alsopublished two cases of beard PPB [4].

Dermatoscopy confirms the absence of hairfollicles in the center of alopecic plaque,perifollicular inflammation and follicularhyperkeratosis characteristic to LPP.

The clinical course of PPB is insidious andslowly progressive over the years with theoccurrence of new plaques and subsequentlywith their confluence. Patients accidentallydiscovers alopecia, these could not find hair onthe comb or hairbrush as Degos noticed [3].Alopecia is definitive, the evolutive processspontaneously stops after a few years [1, 3].Rapid progression is extremely uncommon [5,16].

Histopathologically, the changes present inthis context claim that the PPB remains adiagnosis of exclusion. In early stage lesions amoderate perifollicular lymphocytic infiltrateappears, but the interface area is not affected.Subsequently, the infundibular epitheliumatrophy occurs and the concentric lamellarhyperplasia „in onion“ (en oignon) around the

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dispariþie ulterioarã. În stadiul tardiv se observão fibrozã dermicã longitudinalã cu extinderesubcutanatã pentru ca în final folicululpilosebaceu sã fie distrus în totalitate (fig. 2).Atrofia epidermicã subliniatã de Brocq ºi colab.[5] nu a fost observatã de alþi autori. Persistenþafibrelor elastice în partea inferioarã a folicululuireprezintã un semn valoros în diferenþierea PPBde LEC ºi LPP. Practicarea mai multor biopsii esteuneori necesarã pentru stabilirea diagnosticului.

Imunofluorescenþa directã efectuatã dinmarginea unei leziuni este negativã, putândsurprinde uneori depozite granuloase de IgM de-a lungul infundibulului folicular.

În privinþa diagnosticului diferenþial ladebutul PPB acesta trebuie fãcut cu pelada.Aceasta este o alopecie necicatricialã apãrândorificiile pilare vizibile îndeosebi la dermato-scopie, observându-se ºi prezenþa punctelorgalbene (yellow dots) (fig. 3).

În evoluþie diagnosticul diferenþial trebuiefãcut cu alte alopecii cicatriciale precum:

a) Lupusul eritematos discoid care debu-teazã sub forma de papule ºi plãcieritematoase cu extindere centrifugã ºiformarea de plãci eritematoase deaspectul unor monezi cu prezenþadopurilor foliculare ºi scuamelor ade-rente uneori hiperkeratozice (fig. 4). Înevoluþie eritemul diminueazã treptat cuprezenþa atrofiei, telangiectaziilor,depigmentãrii ºi dispariþiei ostiilor foli-culare. Dermatoscopia evidenþiazã înLEC prezenþa de mega points (globicornoºi) în zonele vechi active, atelangiectaziilor, a micilor puncte roºiifoliculare (în zonele recente), a scuamelorgroase ºi discromice.

b) Lichenul plan pilar se prezintã sub formade plãci mici multifocale cu evoluþiecentrifugã ºi posibilitatea confluãrii lor.Eritemul perifolicular, papulele violaceesau brune ºi hiperkeratoza folicularãspinoasã sunt de asemenea prezente fiindînsoþite de cãderea pãrului ºi prurit (fig.5). Dermatoscopia evidenþiazã inflamaþiarozã peripilarã (aspect de jeleu de fructe)în jurul orificiului pilar ºi descuamareaperipilarã. Examenul anatomopatologicºi imunofluorescenþa cutanatã efectuate

upper portion of the hair follicle. The sebaceousglands are initially reduced in size butsubsequently these may disappear. In the latestage there is a longitudinal dermal fibrosis withsubcutaneous extension and finally the pilo-sebaceous follicle is completely destroyed (fig. 2).Epidermal atrophy noted by Brocq et al. [5] hasnot been remarked by other authors. Thepersistence of elastic fibers in the bottom of thehair follicle is a valuable sign in differentiatingPPB of DLE and LPP. Sometimes multiplebiopsies are required for diagnosis.

Direct immunofluorescence performed fromthe border of a lesion is negative, but sometimesit can reveal IgM granular deposits along thefollicular infundibulum.

Regarding the differential diagnosis, in theearly stage of PPB it must be done with pelada.This is a noncicatricial alopecia, the hair orificesare visible particularly in dermatoscopy, alsoobserving the presence of yellow dots (fig. 3).

In evolution of PPB, the differential diagnosismust be made with other cicatricial alopecia suchas:

a) Discoid lupus erythematosus whichstarts as erythematous papules andplaques with centrifugal extension anderythematous plaques formation withcoin-like appearance, with the presenceof follicular plugs and adherent andsometimes hyperkeratotic scales (fig. 4).In evolution, the erythema with presenceatrophy gradually diminishes withatrophy, teleangiectasias, depigmentationand disappearance of follicular ostium.DLE dermoscopy highlights the presenceof mega points in still active old areas,teleangiectasias, follicular small red spots(in recent areas), thick and dyschromicscales.

b) Lichen planopilaris presents as smallmultifocal plaques with centrifugal evo-lution and possibly plaque confluation.Perifollicular erythema, violaceous orbrown papules and spinous follicularhyperkeratosis are also accompanied byhair loss and itching (fig. 5). Dermato-scopy highlights a pink peripilarinflammation (appearance of fruit jelly)around the hair orifice perifollicular

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Figura 2 – Pseudopelada – aspect histopatologicFigure 2 – Pseudopelade – histopathological appearance

Figura 3 – Pelada scalpului – aspect clinicFigure 3 – Pelada of the scalp – clinical appearance

Figura 1 – Pseudopelada – aspect clinicFigure 1 – Pseudopelade – clinical appearance

Figura 4 – Lupus eritematos discoid al scalpului – aspectclinic

Figure 4 – Discoid lupus erythematosus of the scalp -clinical appearance

Figura 5 – Lichen plan pilar al scalpului – aspect clinicFigure 5 – Lichen planopilaris of the scalp - clinical

appearance

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din marginea unei leziuni ajutã lastabilirea diagnosticului de LPP.

c) Alopecia cicatricialã centralã centrifugãafecteazã în special femeile de rasãneagrã prezentând câteva caracteristiciale PPB. Afecteazã vertexul scalpului prinapariþia de plãci cicatriciale cu extinderecentrifugã simetricã ºi lentã fãrã prezenþade semne inflamatorii. Cicatricea tegu-mentarã este suplã, subþire, de culoareacãrnii. Pe zonele cicatriciale se observãinsule de pãr neafectat. O hiper-pigmentaþie perifolicularã ºi politrichiepot fi observate.

d) Alopecia parvimaculata se caracterizeazãprin apariþia de mici plãci dintre care oparte au evoluþie finalã spre alopeciacicatricialã. Plãcile sunt în general izolateiar numãrul lor este mic. În modparticular alopecia parvimaculata a fostdescrisã la copii sub formã de epidemie[17]. Dupã o serie de autori indivi-dualitatea acestei afecþiuni este discu-tabilã.

e) Alte diagnostice diferenþiale trebuiefãcute cu morfeea, tinea capitis, sifilisulsecundar sau alopecia familialã focalã.

Pacientul trebuie sã fie informat asupraevoluþiei cronice a PPB cu distrugerea foliculilorpiloºi ºi dificultãþilor terapeutice existente.Evoluþia lentã, asimptomaticã, a PPB fãrãprezenþa semnelor inflamatorii pune în discuþiealegerea terapiei ºi posibilitatea urmãririieficienþei acesteia. În schimb în faza activã aevoluþiei bolii evidenþiatã prin „pull test” pozitivºi caracterizatã prin pierderea mare de pãrtratamentul este necesar.

Dermatocorticoizii de nivel 1 (clobetazolpropionat), triamcinolon acetonid (10 mg/ml)injectat intralezional în marginea plãcii, o ºedinþãla interval de 4-8 sãptãmâni sunt cele maiutilizate. ªamponul cu propionat de clobetazolare o indicaþie discutabilã iar tacrolimusul 0,1% înpomadã pare sã nu fie eficient. Minoxidilul 5% arputea duce o ameliorare cosmeticã prinîngroºarea pãrului rãmas [2]. În formele active, lafel ca în LPP, corticoterapia generalã administratãîn dozã de 1 mg/kg urmatã de descreºtereatreptatã a dozelor pe o perioadã de mai multeluni poate bloca procesul inflamator, dar de

desquamation. Histopathological exami-nation and immunofluorescence per-formed from the border’s lesion helpsestablishing the diagnosis of LPP.

c) Central centrifugal cicatricial alopecia isa condition that generally affects Afro-american women presenting somecharacteristics of PPB. It affects the vertexundergoing slowly progressive, centri-fugal scarring without overt inflam-mation. The scarred skin is supple, shinyand flesh-colored. Islands of unaffectedhair may be present within areas of scar.Perifollicular hyperpigmentation andpolytrichia can be observed.

d) Alopecia parvimaculata is characterizedby the appearance of small plaques, someof which have final evolution towardscicatricial alopecia. Plaques are generallyisolated and their number is small. Inparticular alopecia parvimaculata wasdescribed as epidemic in children [17]. Anumber of authors believe that theindividuality of this condition isquestionable.

e) Other differential diagnoses to be madein with morphea, tinea capitis, focalcicatricial alopecia.The patient must be informed on the

chronic evolution of PPB with the destruction ofhair follicles and on the therapeutic difficulties.The slow asymptomatic course of PPB withoutthe presence of inflammatory signs is questioningthe choice of therapy and the possibility offollowing the effectiveness. When the condition isactive, marked by positive pull test andcharacterized by big hair loss treatment isnecessary.

Superpotent topical corticosteroids (clobe-tasol propionate), intralesional triamcinoloneacetonide (10 mg/ml), injection to be made at theborder’s lesion, one every 4-8 weeks are the mostused. Clobetasol propionate shampoo has aquestionable indication and tacrolimus ointment0.1% seems not to be effective. Minoxidil 5%could result in a cosmetic improvement bythickening the remaining hair [2]. In active forms,as well as LPP, general corticotherapy admi-nistered as 1 mg/kg followed by a gradualdecrease of the doses over a period of several

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obicei întreruperea tratamentului este urmatã derecidive. Ciclosporina ºi micofenolatul mofetilpot fi ºi ele utilizate în oprirea procesuluievolutiv. Retinoizii nu au efect, iar eficienþaantipaludicelor de sintezã (hidroxicloroquin îndozã de 200 mg de douã ori pe zi) constatatã de oserie de autori rãmâne discutabilã [18].

Grefele de pãr autolog ºi chirurgia se potefectua dupã doi ani de stabilizare a bolii [2].Thiazolidine-dionele, agoniºti selectivi aireceptorilor activatori ai proliferãrii peroxi-zomilor gama (PPAR-γ) utilizaþi în diabetul de tip2, ar putea fi eficiente în LPP.

În concluzie PPB este o alopecie cicatricialãlimfocitarã cronicã de etiopatogenie necunoscutãimpunând diagnosticul diferenþial cu altealopecii cicatriciale precum LEC ºi LPP. Evoluþialentã, asimptomaticã a PPB fãrã prezenþa semne-lor inflamatorii face ca eficienþa tratamentului sãfie greu de apreciat. Dermatocorticoizii de nivel 1ºi triamcinolonul acetonid injectat intralezionalsunt cele mai utilizate, iar în formele activecorticoterapia generalã este utilizatã la fel ca ºi înLPP.

months can block the inflammatory process, butusually cessation of the treatment is followed byrelapses. Cyclosporine and mycophenolatemofetil can also be used to stop the evolutiveprocess. Retinoids have no effect, and theefficiency of synthetic antimalarial drugs(hydroxychloroquine at a dose of 200 mg twice aday) observed by some authors remainsquestionable [18].

Autologous hair grafts and surgery arepossible after two years of disease stabilization[2]. Thiazolidinediones, peroxisome proliferatoractivated receptor gamma agonists (PPAR-γ)used in the type 2 diabetes could be effective inthe LPP.

In conclusion PPB is a chronic lymphocyticcicatricial alopecia of unknown etiology whichrequires a differential diagnosis with othercicatricial alopecia like DLE and LPP. The slowasymptomatic evolution of PPB, without thepresence of inflammatory signs makes treatmenteffectiveness difficult to assess. Superpotenttopical corticosteroids and intralesionaltriamcinolone acetonide are the most commonlyused, and in active forms general corticosteroid isused like in LPP.

Bibliografie/Bibliography

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89.4. Tan E, Martinka M, Ball N, Shapiro J. Primary cicatricial alopecias: clinicopathology of 112 cases. J Am Acad

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cicatricial alopecia, apparently primary, of pseudopelade type. Ann Dermatol Syphiligr (Paris). 1954 Jan-Feb;81(1):5-26

9. Sperling LC, Solomon AR, Whiting DA. A new look at scarring alopecia. Arch Dermatol. 2000 Feb;136(2):235-42.10. Dawber R. What is pseudopelade? Clin Exp Dermatol. 1992 Sep;17(5):305-6.11. Powell J, Dawber RP. Folliculitis decalvans and tufted folliculitis are specific infective diseases that may lead to

scarring, but are not a subset of central centrifugal scarring alopecia. Arch Dermatol. 2001 Mar;137(3):373-4.12. Braun-Falco O, Imai S, Schmoeckel C, Steger O, Bergner T. Pseudopelade of Brocq. Dermatologica. 1986; 172 (1):

18-23.13. Amato L, Mei S, Massi D, Gallerani I, Fabbri P. Cicatricial alopecia; a dermatopathologic and immunopathologic

study of 33 patients (pseudopelade of Brocq is not a specific clinico-pathologic entity). Int J Dermatol. 2002Jan;41(1):8-15.

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14. Olsen EA, Bergfeld WF, Cotsarelis G, Price VH, Shapiro J, Sinclair R, et al. Workshop on Cicatricial Alopecia.Summary of North American Hair Research Society (NAHRS)-sponsored Workshop on Cicatricial Alopecia,Duke University Medical Center, February 10 and 11, 2001. J Am Acad Dermatol. 2003 Jan;48(1):103-10.

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19. http://www.dxpath.com/histlib/pseudopelade-of-Brocq-histopathology-20503.html. Accesed 15 Sept 2015.

Conflict de interese Conflict of interestNEDECLARATE NONE DECLARED

Adresa de corespondenþã: Prof. univ. dr. Alexandru OanþãAdresã: Braºov, str. Zizinului, nr. 40Telefon: 0268333825Email: [email protected]

Correspondance address: Prof. univ. dr. Alexandru OanþãAddress: Braºov, 40 Zizinului streetPhone: 0268333825Email: [email protected]