PMID- 25558948
OWN - NLM
STAT- MEDLINE
DCOM- 20161014
LR  - 20210331
IS  - 1346-8138 (Electronic)
IS  - 0385-2407 (Print)
IS  - 0385-2407 (Linking)
VI  - 42
IP  - 1
DP  - 2015 Jan
TI  - Update on fogo selvagem, an endemic form of pemphigus foliaceus.
PG  - 18-26
LID - 10.1111/1346-8138.12675 [doi]
AB  - Pemphigus are organ-specific autoimmune diseases, where autoantibodies (mainly 
      immunoglobulin [Ig]G) directed against epidermal targets (glycoproteins of the 
      desmosomal core) are detected. Endemic pemphigus foliaceus or fogo selvagem (FS) 
      is one of the variants of pemphigus foliaceus pemphigus foliaceus that shares the 
      same clinical and immunopathological features of the classic non-endemic 
      pemphigus foliaceus form, including pathogenic IgG (mainly IgG4) autoantibodies 
      directed against the ectodomain of desmoglein 1 (Dsg1), that lead to 
      acantholysis. Pathogenesis of FS is complex, involving genetic, environmental and 
      immunological factors. Human leukocyte antigen (HLA)-DRB1 alleles DRB1*0404, 
      *1402, *1406 or *0102 have been previously identified as risk factors for FS 
      (relative risk, >14). Individuals exposed to hematophagous insects are more 
      susceptible to develop the disease. Non-pathogenic anti-Dsg1 antibodies of the 
      IgG1 subclass, directed against the extracellular 5 domain of Dsg1, are detected 
      in patients in the preclinical stage of the disease, and also in healthy controls 
      living in endemic areas. In counterpart, patients with FS show pathogenic 
      anti-Dsg1 IgG4 autoantibodies that bind the pathogenic extracellular 1 and 2 
      domains of Dsg1, emphasizing the intramolecular epitope-spreading hypothesis. A 
      possible explanation for the development of the autoimmune process would be 
      antigenic mimicry, initiated by environmental stimuli in those genetically 
      predisposed individuals. Characterization of the pathogenesis of FS will allow 
      the development of specific therapeutic targets, and the elucidation of other 
      autoimmune processes.
CI  - (c) 2015 Japanese Dermatological Association.
FAU - Aoki, Valeria
AU  - Aoki V
AD  - Department of Dermatology, University of Sao Paulo Medical School, Sao Paulo, 
      Brazil.
FAU - Rivitti, Evandro A
AU  - Rivitti EA
FAU - Diaz, Luis A
AU  - Diaz LA
CN  - Cooperative Group on Fogo Selvagem Research
LA  - eng
GR  - R01 AR032599/AR/NIAMS NIH HHS/United States
GR  - 2R01AR032599-31/AR/NIAMS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - England
TA  - J Dermatol
JT  - The Journal of dermatology
JID - 7600545
SB  - IM
MH  - Brazil/epidemiology
MH  - *Endemic Diseases
MH  - Humans
MH  - Pemphigus/*epidemiology/etiology
PMC - PMC4496802
MID - NIHMS630478
OTO - NOTNLM
OT  - autoimmunity
OT  - desmoglein
OT  - immunofluorescence
OT  - immunoglobulin G
OT  - pemphigus
EDAT- 2015/01/07 06:00
MHDA- 2016/10/16 06:00
PMCR- 2016/01/01
CRDT- 2015/01/07 06:00
PHST- 2014/09/13 00:00 [received]
PHST- 2014/09/17 00:00 [accepted]
PHST- 2015/01/07 06:00 [entrez]
PHST- 2015/01/07 06:00 [pubmed]
PHST- 2016/10/16 06:00 [medline]
PHST- 2016/01/01 00:00 [pmc-release]
AID - 10.1111/1346-8138.12675 [doi]
PST - ppublish
SO  - J Dermatol. 2015 Jan;42(1):18-26. doi: 10.1111/1346-8138.12675.