Pemphigus foliaceus, brazilianL10.3

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

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Synonym(s)

Brazilian pemphigus; endemic pemphigus foliaceus; Fogo Selvagem; pemphigus brasiliensis

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HistoryThis section has been translated automatically.

Paes-Leme, 1903; Vieira, 1940

DefinitionThis section has been translated automatically.

Variant of Pemphigus foliaceus, endemic to South America.

Occurrence/EpidemiologyThis section has been translated automatically.

  • Endemic in some rural areas along rivers, especially in Brazil, Colombia, El Salvador, Paraguay, Peru, Argentina and Tunisia.
  • Prevalence: up to 5% of the population of endemic areas.

EtiopathogenesisThis section has been translated automatically.

Autoimmune disease with formation of autoantibodies against desmoglein 1. infectious (viral) etiology, associations with frequently occurring insect bites by Simulium sp. ("black fly"; Simulium nigrimanum) as well as reactions against environmental antigens in endemic areas are discussed

ManifestationThis section has been translated automatically.

Especially occurring in children, adolescents or young adults.

LocalizationThis section has been translated automatically.

Face and head, also chest and back; no mucous membrane involvement. Predominantly symmetrical distribution pattern.

Clinical featuresThis section has been translated automatically.

Flat, rapidly bursting bubbles, layered, scaly crusts on a reddened ground. Burning of the skin ("wild fire"), transition to secondary erythroderma possible after years. Polymorphic picture due to simultaneous occurrence of bullous, papillomatous, verruciform, pustular, erythrodermic and hyperpigmented skin changes. Nikolski phenomenon I positive.

HistologyThis section has been translated automatically.

S.u. Pemphigus foliaceus.

Direct ImmunofluorescenceThis section has been translated automatically.

Antiepithelial antibodies against desmoglein 1.

Indirect immunofluorescenceThis section has been translated automatically.

Pemphigus antibody positive, titer curve parallel to the severity of the disease.

TherapyThis section has been translated automatically.

According to the pemphigus foliaceus.

Progression/forecastThis section has been translated automatically.

Mortality: 5%. Cure rate for multi-year corticosteroid therapy: 55%.

Note(s)This section has been translated automatically.

The correct site for taking a biopsy is still a controversial issue. In a study in 47 patients, antibodies were found in over 90% of cases in lesional, perilesional and healthy skin.

LiteratureThis section has been translated automatically.

  1. Abreu-Velez AM (2003) Analyses of autoantigens in a new form of endemic pemphigus foliaceus in Colombia. J Am Acad Dermatol 49: 609-614
  2. Abreu-Velez AM et al (2003) A unique form of endemic pemphigus in northern Colombia. J Am Acad Dermatol 49: 599-608
  3. Chiossi MP et al (2001) Endemic Pemphigus foliaceus ("Fogo selvagem"): a series from the Northeastern region of the State of Sao Paulo, Brazil, 1973-1998 Rev Inst Med Trop Sao Paulo 43: 59-62
  4. Friedman H et al (1995) Endemic pemphigus foliaceus (fogo selvagem) in native Americans from Brazil. J Am Acad Dermatol 32: 949-956
  5. Guillet G et al (1984) Pemphigus bresilien de l ènfant en guyane francaise. Ann. Dermatol. Vernereol. 111: 1087-1092
  6. Kunte C et al (1997) Brazilian pemphigus foliaceus (fogo selvagem). dermatologist 48: 228-233
  7. Paes-Leme C (1903) Contribuicao ao estudo do Tokelau. Doctoral thesis, Rio de Janeiro, Brazil: Facultade de Medicina
  8. Pegas JR et al (2004) Direct immunofluorescence on uninvolved, lesional and perilesional skin in patients with endemic pemphigus foliaceus (fogo selvagem). Med Sci Monit 10: 657-661
  9. Viera JP (1940) Novas contribuições ao estudo do pênfigo foliáceo (fogo selvagem) no Estado de São Paulo. Empresa Gráfica da Revista dos Tribunais (1940)
  10. Viera JP (1942) Pênfigo foliáceo e síndrome de Senear-Uscher. Empresa Gráfica da Revista dos Tribunais (São Paulo)
  11. Warren SJ et al (2000) The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. Cooperative Group on Fogo Selvagem Research. N Engl J Med 343: 23-30
  12. Warren SJ et al (2003) The role of subclass switching in the pathogenesis of endemic pemphigus foliaceus. J Invest Dermatol 120: 104-108

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer