dyskératome verruqueux intéressant deux follicules pileux adjacents: une tumeur inhabituelle

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1 dyskératome verruqueux intéressant deux follicules pileux adjacents: une tumeur inhabituelle Journal : La Tunisie Médicale Manuscript ID : TUNISMED-0-0 Type : ARTICLES ORIGINAUX Date de soumission : /0/0 Auteurs : Hammedi Faten :() Department of Pathology. FattoumaBourguiba Hospital Monastir, Tunisia / Faculté de Medecine de Monastir, Hadhri Rim :() Department of Pathology. FattoumaBourguiba Hospital Monastir, Tunisia / faculté de Médecine de Monastir, Mohamed Mariem :() Department of Dermatology. FattoumaBourguiba Hospital Monastir, Tunisia / Faculté de medecine de Monastir, Ben Abdeljelil Nouha :() Department of Dermatology. FattoumaBourguiba Hospital Monastir, Tunisia / Faculté de Page /

2 Médecine de Monastir, Akari Hayet :() Department of Dermatology. FattoumaBourguiba Hospital Monastir, Tunisia / faculté de medecine de Monastir, Njim Leila :() Department of Pathology. FattoumaBourguiba Hospital Monastir, Tunisia / faculté de medecine de Monastir, Moussa Adnène :() Department of Pathology. FattoumaBourguiba Hospital Monastir, Tunisia / faculté de medecine demonastir, Zakhama Abdelfatteh :() Department of Pathology. FattoumaBourguiba Hospital Monastir, Tunisia / faculté de Médecine de Monastir, Mot clés Dyskératome verruqueux, cuir chevelu, deux follicules adjacents. L auteur correspondant confirme aux noms des co auteurs que : - Tous les auteurs ont contribué à la préparation du manuscrit et sont au courant de son contenu. - Ce manuscrit n a pas été publié ou en cours de soumission dans un autre journal. - Il n y a aucun conflit d intérêt pour chaque auteur avec le contenu du manuscrit. - En cas d acceptation pour publication, les auteurs doivent transférer le copyright du Page /

3 manuscrit au journal. Page /

4 Lettre de motivation Page /

5 Résumé Pré-requis : Le dyskératome verruqueux encore appelé dyskératose folliculaire isolé ou dyskératome folliculaire est une tumeur épithéliale bénigne rare qui se développe aux dépens de l appareil pilo-sébacé. Objectif du travail: Etudier les caractéristiques épidémiologiques anatomo-cliniques de cette entité qui intéresse très rarement deux follicules adjacents. Méthode: Il s agissait d un patient âgé de ans, sans antécédents pathologiques qui consultait au service de dermatologie, pour une papule du cuir chevelu, évoluant depuis ans. Le patient a eu une exérèse large de ce nodule. Résultat: Le diagnostic retenu est celui d un dyskératome verruqueux intréssant deux follicules pileux adjacents. Le patient n a pas présenté de récidive dans les cinq mois qui suivait le diagnostic. Conclusion : Le DV est une tumeur rare. Le traitement de choix consiste en une exérèse chirurgicale avec un pronostic favorable sans récidive. Page /

6 Warty Dyskeratoma in two neighboring follicles: An unusual tumor Auteur correspondant: Ben Abdeljelil Nouha Adresse: laboratoire d'anatomie et de Cytologie Pathologiques. Hôpital Fattouma Bourguiba Monastir. Tunisie Téléphone: nouhaba@yahoo.fr Abstract: Page /

7 Pre Required: Warty dyskeratoma is a benign papulonodular lesion characterized by an endophytic proliferation of squamous epithelium typically occurring in relation to a folliculosebaceous unit and showing prominent acantholytic dyskeratosis.occasionally, the lesions can be multiple. Objective: To study the clinicopathological and epidemiological characteristics of this entity that rarely involves two adjacent follicles. Methods A -year-old man has for one year developped a single small nodule with a hyperkeratotic area on the scalp. Results: Histopathology revealed warty dyskeratoma involving two neighboring follicles. Conclusion: Warty dyskeratoma is a rare lesion that the pathogenesis is still unclear. Key words: Warty dyskeratoma, scalp, two adjoining follicles. Résumé: Pré-requis : Le dyskératome verruqueux encore appelé dyskératose folliculaire isolé ou dyskératome folliculaire est une tumeur épithéliale bénigne rare qui se développe aux dépens de l appareil pilo-sébacé. Page /

8 Objectif du travail: Etudier les caractéristiques épidémiologiques anatomo-cliniques de cette entité qui intéresse très rarement deux follicules adjacents. Méthode: Il s agissait d un patient âgé de ans, sans antécédents pathologiques qui consultait au service de dermatologie, pour une papule du cuir chevelu, évoluant depuis ans. Le patient a eu une exérèse large de ce nodule. Résultat: Le diagnostic retenu est celui d un dyskératome verruqueux intréssant deux follicules pileux adjacents. Le patient n a pas présenté de récidive dans les cinq mois qui suivait le diagnostic. Conclusion : Le DV est une tumeur rare. Le traitement de choix consiste en une exérèse chirurgicale avec un pronostic favorable sans récidive. Mots clé : dyskératome verruqueux, cuir chevelu, deux follicules adjacents. Introduction: Page /

9 Warty dyskeratoma (WD) is an uncommon lesion of the skin that is considered to be associated with the pilosebaceous apparatus. It is a rare, benign, epithelial tumor characterized by foci of acantholytic dyskeratosis []. It usually presents on the sun-exposed skin of the head and neck. Histopathology is important for the diagnosis.solitary lesion arising in two to four follicles have been exceptionally reported [, ].The aims of this paper are to report a new case of an unusual case of WD involving two adjoining follicles, to discuss its differential diagnosis and to describe its follow-up. Case report: We report a -year-old man who presented, to the dermatology department of Fattouma Bourguiba Hospital of Monastir, with a papule of the scalp. The patient s medical and family histories were unremarkable. Physical exam founded a brown hyperkeratotic papule that meseared cm. The diagnostic of pilomatricoma was suggested. The patient underwent a large excision of the mass and the histology demonstrated two well-demarcated cup-shaped epidermal invaginations filled with abundant keratin. These lesions showed acantholysis. This results in suprabasalclefting with formation of villi which protrude into a lacuna. There were typical corps ronds in the thickened granular layer and many acantholytic dyskeratotic cells above the villi. The underlying dermis was infiltrated by lymphocytes and histiocytes. The pathologic diagnosis was WD of the scalp. There is no recurrence or metastasis five months after diagnosis. Discussion: WD or Isolated dyskeratosis follicularis or follicular dyskeratoma was first described by Graham and Helwig [] as isolated Darier s disease in.in, Szymanski Page /

10 [] introduced the name of WD. Because of the rarity of this lesion, most literature about these cases is case reports or a few numbers of patients. It occurs mostly in middle aged to elderly adults. WD frequently arises as a solitary flesh coloured tobrown papules, nodules or cysts with an umbilicated or central keratin plug. Most are -0mm in size. Occasionally, the lesions can be multiple [].To the best of our knowledge, our case represents the third reported case of WD involving two adjoining follicles. The others two lesions were reported by Chon et al [] and Jang et al []. Lesions are commonly asymptomatic but occasionally discharge and bleeding may be encountered.histopathology examination demonstrates a well-demarcated cup-shaped epidermal invagination filled with abundant keratin and acantholysis. This results in suprabasal clefting with formation of villi which protrude into a lacuna. There were typical corps ronds in the thickened granular layer and many acantholytic dyskeratotic cells above the villi. In our case, we founded typical histologic features of WD which involved two neighboring follicles. Differential diagnoses arise with both malignant and benign lesions. In fact, acantholytic dyskeratosisis also observed in Darier s disease, Hailey-Hailey disease, Grover s disease and in some neoplasms such as acantholytic squamous cell carcinoma, actinic keratosis and basal cell carcinoma. However, WD lacks the pleomorphism, nuclear atypia, the hypercellular of these malignant lesions. Although there are some histological similarities with Darier s disease, Hailey-Hailey disease and Grover s disease, deeply penetrating crateriform lesions with villus formation are not associated with these entities and these lesions have a distinctive clinical presentation [,, ]. The pathogenesis of this unusual lesion is still unclear. Some authors have suggested an effect of actinic radiation or a viral infection. Thus, Szymanski [] Page 0/

11 showed the presence of viral DNA and RNA in the epithelial cells. However, Kaddu S et al [] showed no evidence of HPV in cases using PCR. Other authors [,, ] have suggested that WD is most probably of follicular derivation. However, the occurrence of WD in the oral and genital mucosa could not be explained by this theory. For this reason, mucosal variants could have a different derivation. The treatment of choice is local surgical excision [, ]. No recurrences were reported in follow-up studies of WD [,, ], like our case. References: Page /

12 [] Jang EJ, Lee JY, Kim MK, et al. Warty dyskeratoma involving two adjoining follicles. Ann Dermatol. 0 Feb;:-00. [] Tanay A, Mehregan AH. Warty dyskeratoma.dermatologica;:-. [] Graham JH, Helwig EB. Isolated dyskeratosisfollicularis. AMA Arch Derm;:-. [] Szymanski FJ. Warty dyskeratoma; a benign cutaneous tumorresembling Darier s disease microscopically. AMA ArchDerm ;:-. [] Chon HJ, Hong SJ, Kang H, et al. A case of warty dyskeratoma. Korean J Dermatol ;:-. [] Kaddu S, Dong H, Mayer G, et al. Warty dyskeratoma follicular dyskeratoma : analysis of clinicopathologicfeatures of a distinctive follicular adnexal neoplasm. J Am AcadDermatol00; : -. [] Azuma Y, Matsukawa A. Warty dyskeratoma with multiple lesions. J Dermatol ; 0: -. Page /

13 Legends: Fig.: Two well-demarcated cup-shaped epidermal invaginations filled with abundant keratin (HE x 0) Fig. : Note the acantholysis and villi (HE x 00) Fig. : Corps ronds are conspicuous (HE x 00) Page /

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