Endemic pemphigus in the peruvian Amazon: epidemiology and risk factors for the development of complications during treatment

Abstract

BACKGROUNDPemphigus is an autoimmune blistering disease. According to a report, in areas of endemic pemphigus foliaceus (EPF) in Peru there are cases of pemphigus vulgaris with epidemiologic, clinical and histopathologic characteristics similar to those of "endemic pemphigus vulgaris" (EPV) in Brazil. OBJECTIVESTo determine the clinical and epidemiologic characteristics of endemic pemphigus and the risk factors of patients for developing complications during treatment. METHODSA study was carried out from July 2003 to March 2008. The study population was 60 patients with EPF and 7 patients with EPV evaluated in hospitals and clinics in the Peruvian Amazon and Lima. A multivariate analysis was carried out using binary logistic regression. RESULTSThe average age of EPF patients was 31.4 years; 55% were men; 60% presented the generalized clinical variant. Non-compliance with the treatment was seen in 57.1% of the patients. Thirty-five percent presented complications (e.g. pyodermitis and pyelonephritis) during treatment. The risk factors for developing complications during treatment were non-compliance with the treatment and having the generalized clinical form. In the EPV group, the average age was 21.7 years; 71.4% were men. All patients presented with the mucocutaneous clinical variant and the initial presentation consisted of oral mucosa lesions; 71.4% presented complications during treatment, pyodermitis being the most frequent. CONCLUSIONSNon-compliance with the treatment and the generalized clinical form are risk factors for the development of complications during treatment of patients with EPF. Peru indeed has EPV cases with epidemiologic characteristics similar to EPF. Living in a rural area may represent a risk factor for the development of complications during treatment of patients with EPV.

Reviewing putative industrial triggering in pemphigus: cluster of pemphigus in the area near the wastewater treatment plant

“…The role of polyphenolic compounds – tannins, contained in food or water, was suggested in research articles and case reports describing fogo selvagem (“wild fire”) endemic PF [41–45]. Such phenol derivatives include liquid diphenyl sulphide [36] and various synthetic phenolic antioxidants (e.g.…”

Mast cells, Mast/Stem Cell Growth Factor receptor (c-kit/cd117) and IgE may be integral to the pathogenesis of endemic pemphigus foliaceus

“…The roles of IgG autoantibodies and complement in lesional EPF skin has been previously documented [1][2][3][4][5][6][7].…”

“…Endemic pemphigus foliaceus (EPF) is an autoimmune blistering skin disease presenting in well-defined geographic regions [1][2][3][4][5][6][7]. The roles of IgG autoantibodies and complement in lesional EPF skin has been previously documented [1][2][3][4][5][6][7].…”

Antidesmoglein 1 and 3 antibodies in healthy subjects of a population in the Peruvian high amazon

“…Endemic pemphigus foliaceus (EPF) and endemic pemphigus vulgaris (EPV) are the only autoimmune diseases known to occur in delineated areas with an endemic pattern. [1][2][3][4][5][6][7][8][9] The etiology of both diseases remains unknown; however, the action of one or many environmental triggers has been proposed. [10][11][12] A preponderant role has been attributed to the sting of hematophagous insects, particularly the genus Simullium.…”