Anti-plectin pemphigoidL12.0

Clinically, this non-scarring dermatosis differs only slightly from other pemphigoids. It is characterized by the appearance of blisters and erosions in the skin (and mucous membrane?).

In larger unselected collectives of blistering diseases, antibodies against plectin were detected in 3.9% of patients. In most cases other pemphigoid antibodies were also detectable.

Plectin is a structural protein that binds to intermediate filaments. It is detectable in the cytoplasm of almost all cells. This is especially true in muscle and heart muscle cells, epidermal cells, nerve cells and placental cells. Plectin cross-links actin, tubulin and some other intermediate filaments.

In the epidermis, the basal epithelial cells are connected to the underlying basal lamina by the hemidesmosomes.

The connection is established by the intracellular proteins BP230 and plectin. BP230 binds via BP180, plectin via integrin alpha 6 beta 4, which in turn binds to laminin 5 and finally establishes contact with collagen type VII.

Thus, the interaction of plectin with other structural proteins is important for epidermal integrity.

Linear IgG and C3 deposition at the dermo-epidermal junction. As an antigen, plectin is a 500 kDa protein located in the lower parts of the lamina lucida.

1. Buijsrogge JJ et al (2009) Antiplectin autoantibodies in subepidermal blistering diseases. Br J Dermatol 161:762-771.
2. Chan LS (1997) Human skin basement membrane in health and in autoimmune diseases. Front Biosci 2:d343-52.
3. Georgi M et al (2001) Autoantigens of subepidermal bullous autoimmune dermatoses. Dermatologist 52:1079-1089
4. McMillan JR et al (2007) Plectin defects in epidermolysis bullosa simplex with muscular dystrophy. Muscle nerve 35:24-35