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Mediators of Inflammation
Volume 2010, Article ID 350304, 8 pages
Research Article

Possible Implication of Local Immune Response in Darier's Disease: An Immunohistochemical Characterization of Lesional Inflammatory Infiltrate

1Section of Pathological Anatomy, Department of Human Pathology and Oncology, University of Siena, Policlinico Santa Maria alle Scotte, Via delle Scotte, 6, 53100 Siena, Italy
2Section of Dermatology, Department of Clinical Medicine and Immunological Science, University of Siena, 53100 Siena, Italy
3Unit of Rheumatology, Interdepartmental Research Center of Systemic Autoimmune and Autoinflammatory Diseases, University of Siena, Policlinico Le Scotte, 53100 Siena, Italy
4Department of Odontostomatological and Ophthalmological Science, University of Siena, 53100 Siena, Italy

Received 30 March 2010; Accepted 17 May 2010

Academic Editor: Philip W. Wertz

Copyright © 2010 Clelia Miracco et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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 < . 0 5 ) 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.