Tight junctions in Hailey-Hailey and Darier’s diseases


Submitted: 1 October 2009
Accepted: 3 November 2009
Published: 13 November 2009
Abstract Views: 1329
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Authors

  • Laura Raiko Department of Dermatology, University of Turku, Finland.
  • Pekka Leinonen Department of Anatomy and Cell Biology, University of Oulu, Oulu, Finland, Finland.
  • Päivi Hägg Department of Dermatology, University of Oulu, Finland.
  • Juha Peltonen Institute of Biomedicine, Department of Anatomy, University of Turku, Finland.
  • Aarne Oikarinen Department of Dermatology, University of Oulu, Finland.
  • Sirkku Peltonen Department of Dermatology, University of Turku, and Turku University Central Hospital, Finland.
Hailey-Hailey disease (HHD) and Darier’s disease (DD) are caused by mutations in Ca2+-ATPases with the end result of desmosomal disruption and suprabasal acantholysis. Tight junctions (TJ) are located in the granular cell layer in normal skin and contribute to the epidermal barrier. Aberrations in the epidermal differentiation, such as in psoriasis, have been shown to lead to changes in the expression of TJ components. Our aim was to elucidate the expression and dynamics of the TJ proteins during the disruption of desmosomes in HHD and DD lesions. Indirect immunofluorescence and avidin-biotin labeling for TJ, desmosomal and adherens junction proteins, and subsequent analyses with the confocal laser scanning microscope were carried out on 14 HHD and 14 DD skin samples. Transepidermal water loss (TEWL) was measured in normal and lesional epidermis of nine HHD and eight DD patients to evaluate the function of the epidermal barrier in HHD and DD skin. The localization of TJ proteins claudin-1, claudin-4, ZO-1, and occludin in perilesional HHD and DD epidermis was similar to that previously described in normal skin. In HHD lesions the tissue distribution of ZO-1 expanded to the acantholytic spinous cells. In agreement with previous findings, desmoplakin was localized intracellularly. In contrast claudin-1 and ZO-1 persisted in the cell-cell contact sites of acantholytic cells. TEWL was increased in the lesional skin. The current results suggest that TJ components follow different dynamics in acantholysis of HHD and DD compared to desmosomal and adherens junction proteins.

Supporting Agencies

the Finnish Medical Foundation, Finnish Cultural Foundation, Academy of Finland, the Turku University Foundation, the Southwest Finland Hospital District and Northern Ostrobothnia Hospital District, Finland.

Raiko, L., Leinonen, P., Hägg, P., Peltonen, J., Oikarinen, A., & Peltonen, S. (2009). Tight junctions in Hailey-Hailey and Darier’s diseases. Dermatology Reports, 1(1), e1. https://doi.org/10.4081/dr.2009.e1

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