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Pemphigus Erythematosus

Background

The various types of pemphigus include pemphigus erythematosus, pemphigoid, pemphigus vegetans, pemphigus vulgaris, and pemphigus foliaceus.

Pemphigus erythematosus, also known as Senear-Usher syndrome, is an overlap syndrome with features of lupus erythematosus (LE) and pemphigus foliaceus. Pemphigus is demonstrated by acantholysis and immunoglobulin deposits in the interkeratinocyte substance (see the image below).

Direct immunofluorescence microscopy performed on

Direct immunofluorescence microscopy performed on epithelial biopsy specimen obtained from a patient with pemphigus vulgaris detects immunoglobulin G deposits at the epithelial cell surfaces.

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Pérez-Pérez et al recently hypothesized that pemphigus erythematosus is a multiple autoimmune disease.

High doses of UV light are suggested to be the cause of cleavage of the desmoglein-1 ectodomain. As in cases of pemphigus foliaceus the circulating anti–desmoglein-1 antibodies precipitate this cleaved off ectodomain along with the basement membrane zone, resulting in a lupus band–like appearance.

The lupus component of pemphigus erythematosus is demonstrated by circulating antinuclear antibodies (ANA) and sometimes by immunoglobulin and complement deposits at the dermoepidermal junction

For a thorough description and introduction to the possible causes of pemphigus, see the article Pemphigus: An Acronym for a Disease with Multiple Causes “, published by the International Pemphigus Society.

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