Tag Archives: pemphigus

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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 1 (Dsg1).

Objectives  To examine early acantholysis in skin of PF patients at an ultrastructural level.

Methods  Two Nikolsky negative (N-), five Nikolsky positive (N+) and two lesional skin biopsies of immunoserological defined PF patients were studied by light and electron microscopy.

Results  We found no abnormalities in N- PF skin, whereas all 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 non-junctional Dsg1, leading to intercellular widening between desmosomes starting in the lower layers and spreading upwards. Depletion of non-junctional Dsg1 impairs the assembly of desmosomes, resulting in hypoplastic 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.

http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2133.2012.11173.x/abstract

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IgA pemphigus includes subcorneal pustular dermatosis (SPD)-type and intraepidermal neutrophilic IgA dermatosis (IEN)-type. Cases of IgG/IgA pemphigus have recently been documented1. Nonetheless, individual reports of IgA pemphigus indicate considerable heterogeneity.

http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2133.2012.11127.x/abstract

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  This article reviews the use of MMF for the treatment of several bullous conditions, and assesses the evidence gathered from clinical trials and case series. According to numerous case series, MMF could be of value in treating refractory disease. The few randomized clinical trials conducted to date of patients with pemphigus and bullous pemphigoid report a similar efficacy for MMF to other immunosuppressants. (Source: Immunology and Allergy Clinics of North America)

from MedWorm: Pemphigus http://www.medworm.com/index.php?rid=6018247&cid=c_297_3_f&fid=33229&url=http%3A%2F%2Fwww.immunology.theclinics.com%2Farticle%2FPIIS0889856112000252%2Fabstract%3Frss%3Dyes