Funding sources None.
CLINICAL AND LABORATORY INVESTIGATIONS
Ultrastructure of acantholysis in pemphigus foliaceus re-examined from the current perspective
Article first published online: 5 OCT 2012
© 2012 The Authors. BJD © 2012 British Association of Dermatologists 2012
British Journal of Dermatology
Volume 167, Issue 6, pages 1265–1271, December 2012
How to Cite
van der Wier, G., Jonkman, M.F., Pas, H.H. and Diercks, G.F.H. (2012), Ultrastructure of acantholysis in pemphigus foliaceus re-examined from the current perspective. British Journal of Dermatology, 167: 1265–1271. doi: 10.1111/j.1365-2133.2012.11173.x
Conflicts of interest None declared.
- Issue published online: 26 NOV 2012
- Article first published online: 5 OCT 2012
- Accepted manuscript online: 26 JUL 2012 11:37PM EST
- Accepted for publication 20 July 2012
Background Pemphigus foliaceus (PF) is a chronic cutaneous autoimmune blistering disease that is characterized by superficial blistering of the skin, and according to the current perspective is caused by autoantibodies directed against desmoglein (Dsg) 1.
Objectives To examine early acantholysis in the skin of patients with PF at an ultrastructural level.
Methods Two Nikolsky-negative (N−), five Nikolsky-positive (N+) and two lesional skin biopsies from immunoserologically defined patients with PF were studied by light and electron microscopy.
Results We found no abnormalities in N− PF skin, whereas all the N+ skin biopsies displayed intercellular widening between desmosomes, a decreased number of desmosomes and hypoplastic desmosomes in the lower epidermal layers. Acantholysis was present in two of five N+ biopsies, but only in the upper epidermal layers. The lesional skin biopsies displayed acantholysis in the higher epidermal layers. Hypoplastic desmosomes were partially (pseudo-half-desmosomes) or completely torn off from the opposing cell.
Conclusion We propose the following mechanism for acantholysis in PF: initially PF IgG causes a depletion of nonjunctional Dsg1, leading to intercellular widening between desmosomes starting in the lower layers and spreading upwards. Depletion of nonjunctional Dsg1 impairs the assembly of desmosomes, resulting in hypoplastic desmosomes and a decreased number of desmosomes. In addition, antibodies might promote disassembly of desmosomes. In the upper layers of the epidermis, where Dsg3 is not expressed and cannot compensate for Dsg1 loss, ongoing depletion of Dsg1 will finally result in a total disappearance of desmosomes and subsequent acantholysis.