keratoacantoame multiple în asociere cu lupus eritematos cronic

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<ul><li><p>247</p><p>KERATOACANTOAME MULTIPLE N ASOCIERE CULUPUS ERITEMATOS CRONIC PREZENTARE DE CAZ</p><p>MULTIPLE KERATOACANTHOMAS ASSOCIATED WITHCHRONIC LUPUS ERYTHEMATOSUS CASE PRESENTATION</p><p>VASILE BENEA*, MIRCEA TAMPA*,**, DIANA LEAHU*, ALICE RUSU*, CRISTINA RILEANU*,MIHAELA ANCA BENEA*, SIMONA ROXANA GEORGESCU*,**</p><p>* Clinica de Dermatologie, Spitalul clinic de Boli Infecioase i Tropicale Victor Babe Bucureti.Dermatology Clinic, Victor Babe Clinical Hospital of Infectious and Tropical Diseases, Bucharest.</p><p>** Universitatea de Medicin i Farmacie Carol Davila, Bucureti.Carol Davila University of Medicine and Pharmacy, Bucharest.</p><p>Rezumat</p><p>Keratoacantomul este o tumor cutanat caracterizatprin cretere rapid i involuie spontan, ce apare maifrecvent pe zonele fotoexpuse ale persoanelor de vrstemijlocii i btrni.</p><p>Keratoacantoamele multiple au fost descrise ca parte asindroamelor genetice (keratoacantoame eruptive gene-ralizate tip Grzybowsky, keratoacantoame multiplediseminate tip Ferguson-Smith, keratoacantoame tipWitten-Zak), ca urmare a diferitelor tipuri de traumatisme,expunerii ndelungate la carcinogeni chimici (gudroane,rini) sau secundar altor dermatoze (post herpes zoster,lupus eritematos discoid, psoriazis, pemfigus foliaceu,lichen plan, dermatit atopic, acnee conglobat, vitiligoetc.).</p><p>Prezentm cazul unei paciente n vrst de 36 de anicare s-a prezentat n clinic pentru multiple leziuni cusuprafaa exofitic, verucoas, asociate cu plci iplacarde eritematoase i hipopigmentate, localizate pezone fotoexpuse, debutate n urm cu aproximativ 18 luni.</p><p>S-au efectuat biopsii din ambele tipuri de leziuni careau pus n eviden modificri de tip keratoacantom,respectiv lupus discoid.</p><p>Sub tratament cu dermatocorticoizi de poten medie(pentru lupus), creme keratolitice i crioterapie (pentru</p><p>Summary</p><p>Keratoacanthoma is a cutaneous lesion, characterisedby rapid growth and spontaneous involution, occurringmost commonly on the sun-exposed skin areas of middle-aged and elderly people.</p><p>Multiple keratoacanthomas were described as part ofgenetic syndromes (generalised eruptive keratoacanthomasof Grzybowski, disseminated multiple keratoacanthomas ofthe Ferguson-Smith type, keratoacanthomas of Witten andZak) as a result of different types of traumas, prolongedexposure to chemical carcinogens (tars, resins) orsecondary to other dermatoses (post-herpes zoster, discoidlupus erythematosus, psoriasis, pemphigus foliaceus,lichen planus, atopic dermatitis, acne conglobata, vitiligoetc.).</p><p>We present the case of a 36-year-old female patient,who came to the clinic for multiple lesions with anexophytic verrucous surface, associated witherythematous and hypopigmented plaques located on thesun-exposed areas, which had started approximately 18months before.</p><p>Biopsies were performed for both types of lesions thatevidenced keratoacanthoma-type changes and respectivelydiscoid lupus.</p><p>Under treatment with medium potency dermo-corticoids (for lupus), and keratolytic creams and</p><p>CAZURI CLINICECLINICAL CASES</p></li><li><p>248</p><p>DermatoVenerol. (Buc.), 58: 247-262</p><p>Introducere</p><p>Keratoacantomul reprezint o neoplazie acelulelor spinoase keratinizante, cu origineaprobabil la nivelul foliculului pilosebaceu,caracterizat prin cretere rapid i regresiespontan.(1)A fost descris pentru prima dat deSir Jonathan Hutchinson in anul 1889, ca ulcercrateriform al feei (2) i de atunci continu s fieo surs de controverse n ceea ce privetepatogeneza i opiunile terapeutice.(3)Termenulde keratoacantom a fost introdus de Freudenthalof Wroclaw la sfaritul anilor 1940, datoritacantozei considerabile observat n tumor.(2,4) </p><p>Dei cauza acestor leziuni este necunoscut,exist cazuri de keratoacantoame aprute dupdiverse tipuri de traumatisme sau secundar altordermatoze.(5)</p><p>Prezentare de caz</p><p>Prezentm cazul unei paciente n vrst de 36ani care s-a prezentat n clinic pentru multipleleziuni keratozice extensive nsoite de plci iplacarde eritematoase i/sau hipopigmentate,localizate la nivelul zonelor fotoexpuse (mini,fa, membre superioare), cu evoluie deaproximativ 18 luni.</p><p>Istoricul familial a fost negativ pentru bolidermatologice. De asemenea, pacienta a negatcontactul cu substane chimice.</p><p>Examenul clinic a evideniat retromandibularun nodul hemisferic, bine delimitat, cu ulceraiecentral crateriform, nconjurat de o zoneritematoas, la nivelul buzelor cheilit, iar lanivelul feei dorsale a minilor i feei anterioarea antebraului drept plci i placarde binedelimitate, cu margini neregulate, de mrimivariate (civa milimetri pn la 6 cm) cusuprafaa exofitic, verucoas (fig. 1-3). Presternal(fig. 4) i la nivelul membrelor superioare au fostpuse n eviden plci i placarde eritematoasei/sau hipopigmentate, cu margini neregulate,neuniform colorate, cu zone atrofice.</p><p>Introduction</p><p>Keratoacanthoma is a neoplasia of thekeratin-producing squamous cells, likely tooriginate from the pilosebaceous follicle,characterised by rapid growth and spontaneousregression. (1) It was first described by SirJonathan Hutchinson in 1889 as a crateriformulcer of the face (2) and it has been a source ofcontroversy ever since with respect to thepathogenesis and therapeutic options. (3) Theterm keratoacanthoma was coined byFreudenthal of Wroclaw in the late 1940s due tothe considerable acanthosis observed in thetumour. (2, 4) </p><p>Although the cause of these lesions isunknown, there are cases of keratoacanthomasoccurring after various types of trauma orsecondary to other dermatoses. (5)</p><p>Case presentation</p><p>We present the case of a 36-year-old femalepatient who came to the clinic for multipleextensive keratotic lesions accompanied byerythematous and/or hypopigmented plaqueslocated on the sun-exposed areas (hands, face,upper limbs), with an evolution of approximately18 months.</p><p>The family history was negative for skindiseases. The patient also denied any contactwith chemicals.</p><p>The clinical examination revealed ahemispherical nodule in the retromandibularregion, which was well-defined with a centralcrateriform ulceration, surrounded by anerythematous area. The patient also presentedcheilitis and some well-defined plaques on thedorsal side of the hands and the anterior side ofthe right forearm, with irregular borders andvarious sizes (few millimetres to 6 cm) with anexophytic verrucous surface (Fig. 1-3).Erythematous and/or hypopigmented plaques,with irregular borders and uneven colour, with</p><p>keratoacantoame), evoluia a fost favorabil, cu remisiuneaparial a leziunilor.</p><p>Cuvinte cheie: keratoacantoame multiple, lupusdiscoid.</p><p>cryotherapy (for keratoacanthomas), the evolution wasfavourable, with partial remission of lesions.</p><p>Keywords: multiple keratoacanthomas, discoid lupus.</p><p>Intrat n redacie: 16.10.2013</p><p>Acceptat: 11.11.2013</p><p>Received: 16.10.2013</p><p>Accepted: 11.11.2013</p></li><li><p>249</p><p>DermatoVenerol. (Buc.), 58: 247-262</p><p>Nu a fost observat prezena altor leziunicutanate, mucoase sau unghiale.</p><p>Investigaiile de laborator efectuate au fost nlimite normale iar serologia pentru HIV a fostnegativ.</p><p>Examenul histopatologic din leziunile de lanivelul feei dorsale a minii stngi a relevat</p><p>atrophic areas, were evidenced on the presternalarea (Fig. 4) and the upper limbs.</p><p>The presence of other cutaneous, mucosal orungual lesions was not observed.</p><p>The laboratory investigation results werewithin the normal limits and the HIV serologywas negative.</p><p>Fig. 1. Plci i placarde verucoase i hipopigmentate pe faa dorsal a minii drepte</p><p>Fig. 1. Plates and plaques and hypopigmented warty on the back of the right hand</p><p>Fig. 2. Plci i placarde verucoase i hipopigmentate pe faa dorsal a minii stngi</p><p>Fig. 2. Plates and plaques and hypopigmented warty on the back of the left hand</p><p>Fig. 4. Plci hipopigmentate i eritematoase situatepresternal</p><p>Fig. 4. Hypopigmented and erythematous plates locatedpresternal</p><p>Fig. 3. Plci i placarde verucoase i hipopigmentate lanivelul minii i articulaiei radiocarpiene drepte</p><p>Fig. 3. Plates and plaques and hypopigmented warty handand of the right radiocarpal joint</p></li><li><p>250</p><p>DermatoVenerol. (Buc.), 58: 247-262</p><p>modificri caracteristice lupusului eritematosdiscoid: ngroarea membranei bazale cu afectarevacuolar n stratul celular bazal, dopurikeratozice foliculare, hiperkeratoza i atrofiaepidermului i infiltrat limfocitar perivascular(fig. 5). Modificrile histopatologice ale nodu-lului (fig. 6) au inclus o arhitectur crateriform,cu hiperkeratoz marcat, acantoz i papilo-matoz. Celule epiteliale atipice mari ce prezinto citoplasm eozinofilic caracteristic lucioas,nconjoar centrul umplut cu material cornificat.</p><p>Datele clinice coroborate cu cele de laboratorau stabilit diagnosticul de keratoacantoameverucoase multiple asociate cu lupus eritematoscronic discoid.</p><p>Sub tratament cu dermatocorticoizi depoten medie (pentru lupus), creme keratoloticei crioterapie (pentru keratoacantoame), evoluiaa fost lent favorabil (fig. 7), cu regresiuneparial a leziunilor dup dou luni.</p><p>Ulterior, pacienta nu s-a mai prezentat nclinic pentru reevaluare i continuareatratamentului.</p><p>Discuii</p><p>Epidemiologie</p><p>Dei a fost raportat la toate grupele de vrst,keratoacantomul solitar apare rareori la pacieniisub 20 de ani, vrful incidenei fiind ntre 50 i 69de ani. Studiile au artat c distribuia pe sexeeste aproximativ egal, cu o uoar prepon-deren la sexul masculin. Adevrata inciden a</p><p>The histopathological examination of thelesions on the dorsal side of the left handrevealed changes characteristic of discoid lupuserythematosus: basement membrane thickeningwith vacuolar alteration of the basal cell layer,follicular keratotic plugs, hyperkeratosis andatrophy of the epidermis and perivascularlymphocytic infiltrate (Fig. 5). Thehistopathological changes in the nodule (Figure6) included a crateriform architecture, withmarked hyperkeratosis, acanthosis and papillo-matosis. Large atypical epithelial cells showingtypical glassy eosinophilic cytoplasm sur-rounded the core filled with cornified material.</p><p>The clinical data in conjunction with thelaboratory data led to the diagnosis of multipleverrucous keratoacanthomas associated withchronic discoid lupus erythematosus.</p><p>The evolution was slowly favourable (Fig. 7)under treatment with medium potencydermocorticoids (for lupus), and keratolyticcreams and cryotherapy (for keratoacanthomas),with partial regression of the lesions after twomonths.</p><p>The patient did not come back later to theclinic for re-evaluation and further treatment.</p><p>Discussions</p><p>Epidemiology</p><p>Although reported in all age groups, thesolitary keratoacanthoma is rare in patients lessthan 20 years old, as the peak incidence is</p><p>Fig. 5. Examen histopatologic - lupus eritematos cronicFig. 5. Histopathology - chronic lupus erythematosus</p><p>Fig. 6. Examen histopatologic - keratoacantomFig. 6. Histopathology - keratoacanthoma</p></li><li><p>251</p><p>DermatoVenerol. (Buc.), 58: 247-262</p><p>keratoacantoamelor nu este ns cunoscut, nprincipal din cauz c unele leziuni regreseazspontan, i, prin urmare, nu mai ajung sa fietratate de medici.</p><p>Incidena formelor multiple i particulare dekeratoacantoame rmne sczut. Tipul familialde keratoacantoame multiple, eruptive, FergusonSmith poate debuta n adolescen sau chiar mai devreme, cu o inciden pe sexe de B:F = 3:1, spre deosebire de keratoacantoameleeruptive tip Grzybowski care au o distribuieegal pe sexe (B:F = 1:1).(2,4,6).</p><p>Etiopatogenie</p><p>Etiologia keratoacantoamelor rmne necu-noscut. Au fost descrii multipli factori implicain patogeneza keratoacantomului. Apariialeziunilor cu predilecie pe zonele fotoexpuse, nxeroderma pigmentosum i la cei care au urmattratament PUVA sau fototerapie cu UVB (7,8)sugereaz c expunerea cronic la raze UV poatereprezenta un factor de risc important n apariiakeratoacantoamelor. Se pare c PUVA accelereazdezvoltarea leziunilor, dar s-a demonstrat criscul de a dezvolta keratoacantoame duptratamentul PUVA este mai mic dect cel de adezvolta carcinoame spinocelulare. </p><p>Aparenta derivare a keratoacantoamelor dinfoliculii piloi nu poate explica apariia acestorleziuni la nivelul mucoaselor. S-a speculat cleziunile de la nivelul mucoasei bucale sedezvolt din glande sebacee ectopice sau dinstratul epitelial superficial.(9)</p><p>S-a bnuit de mult timp implicarea carcino-genilor chimici (gudroane, rini, insecticide,</p><p>between 50 and 69 years old. Studies have shownthat the gender distribution is approximatelyequal, with a slight predominance in males. Butthe true incidence of keratoacanthomas isunknown, mainly because some lesions regressspontaneously and therefore do not get to betreated by doctors.</p><p>The incidence of the multiple and particularforms of keratoacanthomas remains low. TheFerguson-Smith familial type of multipleeruptive keratoacanthomas may begin inadolescence or even earlier, with a genderincidence of M:F = 3:1, unlike eruptivekeratoacanthomas of Grzybowski that have equalgender distribution (M:F = 1:1). (2, 4, 6)</p><p>Etiopathogeny </p><p>The etiology of keratoacanthomas remainsunknown. Multiple factors involved in thepathogenesis of keratoacanthomas have beendescribed. The occurrence of lesions especially insun-exposed areas, in xeroderma pigmentosumand in people who received PUVA treatment orUVB phototherapy (7, 8) suggests that chronicexposure to UV rays may be an important riskfactor in the development of keratoacanthomas.It seems that PUVA accelerates the developmentof lesions, but it has been demonstrated that therisk of developing keratoacanthomas after PUVAtreatment is lower than that of developingsquamous cell carcinomas. </p><p>The apparent derivation of keratoacanthomasfrom hair follicles cannot explain the occurrence ofthese lesions in the mucous membranes. It hasbeen speculated that oral mucosal lesions</p><p>Fig. 7. Aspect clinicdup o sptmn detratamentFig. 7. Clinical aspectafter one week oftreatment</p></li><li><p>252</p><p>DermatoVenerol. (Buc.), 58: 247-262</p><p>uleiuri minerale i fumatul) n dezvoltareakeratoacantoamelor. Aceast ipotez a fostsusinut de inducerea keratoacantoamelor laoareci prin aplicaii de derivai de gudron dar iprin incidena crescut a acestor leziuni lapersoanele care lucreaz cu petrol i la mineri.(9)</p><p>De asemenea, att traumatismele (grefele depiele, vaccinurile, tatuajele, peelingul, arsuriletermice, radioterapia, crioterapia, fototerapia,punciile arteriale etc.) (5), ct i alte dermatoze(dermatita de staz, lupus eritematos discoid,lichenul plan, vitiligo, herpes zoster, erupiipost-medicamentoase, eritem polimorf, pso-riazis, dermatita seboreic, radiodermita,xeroderma pigmentosum, foliculita, acneeaconglobat, miliaria etc) au fost asociate cuapariia keratoacantoamelor solitare sau mul-tiple (10,11).</p><p>Rolul imunosupresiei n patogenezakeratoacantoamelor nu este pe deplin cunoscut.Exist comunicri ce menioneaz rolultransplantului de mduv, tratamentelor cuciclosporin, infliximab sau sorafenib (12) i ainfeciei HIV/SIDA ca promotori.(13)</p><p>Factorii genetici se pare c joac un rolimportant n tipurile familiale de kerato-acantoame (n keratoacantoamele multiple tipFerguson Smith gena responsabil a fostlocalizat pe cromozomul 9q22-q23, iar nsindromul Muir-Torre genele afectate suntMLH1, MSH2 i MSH 6 ); (2,4,6, 14) Rolul HPVn dezvoltarea keratoacantoamelor nu a fost pedeplin stabilit...</p></li></ul>