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Pemphigus-like antibodies in a patient with cicatricial pemphigoid

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Abstract

Immunofluoroscent testing has become a valuable adjunct to histopathologic studies in the diagnosis of the bullous dermatoses.1–3 In pemphigus, the acantholytic intraepithelial bullae in the stratifield squamous epithelia of the skin and mucous membranes associated with deposits of anti-intercellular substances (ICS) antibodies are detectable by direct immunofluorescence (DIF).4 In pemphigoid, including the bullous and cicatricial forms (benign mucous membrane pemphigoid), nonacantholytic subepithelial bullae in the stratified squamous epithelia of the skin and mucous membranes associated with deposits of antibasement membrane (BM) antibodies are detectable by DIF.5

Circulating anti-ICS antibodies in the serum of pemphigus patients can be detected by indirect immunofluorescence (IIF).4 Like pemphigus, bullous pemphigoid is also characterized by circulating anti-BM antibodies detectable in the serum by IIF. However, circulating anti-BM antibodies are seen much less frequently in cicatricial pemphigoid.5

True pemphigus anti-ICS and true pemphigoid anti-BM antibodies are believed to be disease-specific. However, there have been increasing reports of pemphigus-like anti-ICS antibodies in patients with other clinical conditions, including severe thermal burns,6–9 abnormally high isohemagglutinin titers,10–12 and certain bullous drug eruptions.13–18 Pemphigus-like anti-ICS antibodies have been detected in patients with bullous dermatoses other than pemphigus19–21 including three patients with cicatricial pemphigoid.

In this report, we present a patient with the clinical history and physical findings of cicatricial pemphigoid with a pemphigus-like anti-ICS antibody in the serum detected by IIF.

Case report

A 78-year-old white man was referred to the Department of Thoracic and Cardiovascular Surgery at The Cleveland Clinic Foundation in September 1978 . . .


 

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