title: Possible implication of local immune response in Darier's disease: an immunohistochemical characterization of lesional inflammatory infiltrate.
abstract: Cell-mediated immunity is considered to be normal in Darier's Disease (DD), an inherited skin disorder complicated by skin infections. To date, there are no investigations on the local inflammatory infiltrate in DD skin lesions. In this immunohistochemical study we characterized and quantified it, making comparisons with two other inflammatory skin disorders, that is, pemphigus vulgaris (PV) and lichen ruber planus (LRP), and with the normal skin (NSk). We found a significant (P < .05) decrease of CD1a+ Langerhans cells (LCs) in DD, compared to PV, LRP, and NSk, and of CD123+ plasmacytoid dendritic cells (pDCs), compared to PV and LRP. We hypothesize that the genetic damage of keratinocytes might result in a loss of some subsets of dendritic cells and, consequently, in an impaired local immune response, which might worsen the infections that inevitably occur in this disease.
authors.affiliation: Section of Pathological Anatomy, Department of Human Pathology and Oncology, University of Siena, Policlinico Santa Maria alle Scotte, Via delle Scotte 6, 53100 Siena, Italy. miracco@unisi.it
authors.forename: Clelia
authors.initials: C
authors.lastname: Miracco
authors.forename: Francesco
authors.initials: F
authors.lastname: Pietronudo
authors.forename: Vasileios
authors.initials: V
authors.lastname: Mourmouras
authors.forename: Michele
authors.initials: M
authors.lastname: Pellegrino
authors.forename: Monica
authors.initials: M
authors.lastname: Onorati
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Mastrogiulio
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
authors.forename: Pietro
authors.initials: P
authors.lastname: Luzi
mesh_terms: D000328:Adult; D018949:Antigens, CD1 / Q000276:immunology; D007644:Darier Disease / Q000276:immunology* / Q000473:pathology*; D003713:Dendritic Cells / Q000276:immunology; D005260:Female; D006801:Humans; D053650:Interleukin-3 Receptor alpha Subunit / Q000276:immunology; D007801:Langerhans Cells / Q000276:immunology; D008010:Lichen Planus / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000473:pathology; D012867:Skin / Q000166:cytology / Q000276:immunology* / Q000473:pathology*
chemical_list: D018949:Antigens, CD1; C419393:CD1a antigen; C115022:IL3RA protein, human; D053650:Interleukin-3 Receptor alpha Subunit