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Clin Exp Dermatol. 2013 Aug;38(6):594-600. doi: 10.1111/ced.12029. Epub 2013 May 21.

Endemic pemphigus foliaceus in the Peruvian Amazon.

Author information

1
Department of Dermatology, Virginia Commonwealth University, Richmond, VA, USA. aortegaloayza2@mcvh-vcu.edu

Abstract

BACKGROUND:

Endemic pemphigus foliaceus (EPF) is an organ-specific blistering disease of the epidermis characterized by the presence of IgG autoantibodies, specifically desmoglein (Dsg)1. This condition has been reported particularly in Brazil, Colombia, Tunisia and Peru.

AIM:

To characterize the humoral response against Dsg1 and Dsg3 autoantibodies of patients with EPF from the Peruvian Amazon region.

METHODS:

Blood samples were collected from 16 patients with a clinical diagnosis of EPF, and tested using indirect immunofluorescence (IIF), immunoprecipitation and ELISA (for IgG and its subclasses against Dsg1 and IgG against Dsg3).

RESULTS:

Autoantibodies against the intercellular spaces were detected by IIF in 82.5% and 87.5% of patients, using normal human skin and monkey oesophagus, respectively. Sera from all patients immunoprecipitated recombinant Dsg1, and three serum samples immunoprecipitated recombinant Dsg3 (6.25%). Using ELISA, anti-Dsg1 antibodies were detected in 13 patients (81.25%), and both IgG1 and IgG2 antibodies against Dsg1 in 12 patients (75%). All patients were positive for IgG4 autoantibodies, and only one patient was positive for IgG3 autoantibodies (6.25%). Anti-Dsg3 antibodies were detected in five patients (31.25%).

CONCLUSIONS:

EPF from Peru shares epidemiological, clinical and immunological characteristics with other forms of EPF that have been described in South America.

PMID:
23692307
DOI:
10.1111/ced.12029
[Indexed for MEDLINE]
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