Protection against pemphigus foliaceus by desmoglein 3 in neonates

Article Abstract:

Newborn babies of mothers with pemphigus foliaceus may be protected from the disease because their skin has different characteristics than adult skin. Pemphigus foliaceus is a skin disease caused by antibodies to a skin protein called desmoglein 1. In adults, desmoglein 1 occurs in the surface of the skin whereas another protein called desmoglein 3 occurs in the deeper layers. In the fetus, desmoglein 3 occurs on the surface, and seems to compensate for desmoglein 1 proteins that have been damaged by antibodies. This was demonstrated by a study in mice.

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Paraneoplastic pemphigus: an autoimmune mucocutaneous disease associated with neoplasia

Article Abstract:

Pemphigus, which derives its name from the Greek word for blister, is an autoimmune disease which is recognized in two major forms: pemphigus vulgaris and pemphigus foliaceus. These diseases result from the activity of specific autoantibodies which interfere with normal cell-to-cell adhesion in the skin. The proteins which are bound by the antibodies of both forms of pemphigus have been identified, and both form complexes with plakoglobin, a component of desmosomal and adherens junctions between cells. Antibodies from patients with pemphigus vulgaris bind a 130-kiloDalton protein, while antibodies from pemphigus foliaceus bind a 160-kiloDalton protein. Occasionally, cases of pemphigus are seen which do not fall neatly into either of these two categories. These atypical cases were found to be associated with neoplastic disease, most often lymphoma but not uncommonly with thymoma, leukemia, and other cancers. In this detailed investigation of five patients with atypical pemphigus associated with neoplastic disease, the researchers have confirmed the autoimmune nature of the atypical disorder and have identified a unique complex of four proteins which was recognized by the sera of all five patients, indicating that atypical pemphigus is indeed a distinct clinical entity. One of the most straightforward methods for determining the proteins recognized by antisera is immunoprecipitation. In this method, extracts from radioactively labelled cells are reacted with the antibodies, and anything binding to the antibodies is effectively fished out of the complex mixture. The recovered material may be visualized by molecular weight on an electrophoretic gel. If the antibody binds to a component of a protein complex, all members of the complex will be fished out together and appear in the gel as multiple bands. This is exactly what is seen in the atypical cases of pemphigus. Protein bands of 250, 230, 210, and 190 kiloDaltons were observed after gel electrophoresis using serum from each of the five patients. The authors suggest that this distinct form of pemphigus be called paraneoplastic pemphigus due to its association with neoplastic disease. (Consumer Summary produced by Reliance Medical Information, Inc.)

Analysis, Autoantibodies, Complications and side effects, Physiological aspects, Skin diseases, Tumors, Pemphigus foliaceus

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Pemphigus, bullous impetigo, and the staphylococcal scalded-skin syndrome

Article Abstract:

A molecular pathophysiology of pemphigus is explained to determine the mechanism of the formation of blisters in bullous impetigo and staphylococcal scalded-skin syndrome and to improve on the diagnosis and treatment of it. It is found that manipulation of T cells that are engaged in the control of a variety of physiologic and pathologic immune responses provides a promising option for the treatment of pemphigus.

Care and treatment, Diagnosis, Disease/Disorder overview

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