title: Neural system antigens are recognized by autoantibodies from patients affected by a new variant of endemic pemphigus foliaceus in Colombia.
abstract: BACKGROUND
  Endemic pemphigus foliaceus (EPF), is also known as "fogo selvagem" or "wild fire," reflecting the intense burning sensation of the skin reported by patients with this disease. Based on this finding, we tested for neural autoreactivity in patients affected by a new variant of EPF (El Bagre-EPF).
  
  
  METHODS
  We tested 20 El Bagre-EPF patients, 20 normal controls from the endemic area, and 20 age- and sex-matched normal controls from outside the endemic area. We tested for autoreactivity to several immunoglobulins and complement. Both human skin and bovine tail were used as antigens.
  
  
  RESULTS
  We detected autoreactivity to neural structures, mechanoreceptors, nerves, perineural cell layers of the arachnoid envelope around the optic nerve, brain structures, and to neuromuscular spindles; these structures colocalized with several neural markers. The patient antibodies also colocalized with desmoplakins 1 and 2, with the armadillo repeat protein deleted in velo-cardio-facial syndrome and with p0071 antibodies. Autoreactivity was also found associated with neurovascular bundles innervating the skin, and immunoelectron microscopy using protein A gold against patient antibodies was positive against the nerve axons. Paucicellularity of the intraepidermal nerve endings and defragmentation of the neural plexus were seen in 70% of the cases and not in the controls from the endemic area (p<0.005). Neuropsychological and/or behavioral symptoms were detected in individuals from the endemic area, including sensorimotor axonal neuropathy.
  
  
  CONCLUSIONS
  Our findings may explain for the first time the "pose of pemphigus," representing a dorsiflexural posture seen in EPF patients vis-a-vis the weakness of the extensor nerves, and furthermore, the autoreactivity to nerves in EPF could explain the "burning sensation" encountered in EPF disease.
authors.affiliation: Georgia Dermatopathology Associates, 1534 North Decatur Rd. NE, Suite 206, Atlanta, GA 30307-1000, USA. abreuvelez@yahoo.com
authors.forename: Ana Maria
authors.initials: AM
authors.lastname: Abreu-Velez
authors.forename: Michael S
authors.initials: MS
authors.lastname: Howard
authors.forename: Hong
authors.initials: H
authors.lastname: Yi
authors.forename: Weiqing
authors.initials: W
authors.lastname: Gao
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
authors.forename: Hans E
authors.initials: HE
authors.lastname: Grossniklaus
mesh_terms: D000328:Adult; D000818:Animals; D000941:Antigens / Q000032:analysis / Q000276:immunology* / Q000378:metabolism; D001323:Autoantibodies / Q000032:analysis / Q000276:immunology* / Q000378:metabolism; D001369:Axons / Q000276:immunology / Q000473:pathology; D018959:CD57 Antigens / Q000235:genetics / Q000276:immunology / Q000378:metabolism; D016022:Case-Control Studies; D002417:Cattle; D002831:Choroid Plexus / Q000187:drug effects / Q000276:immunology* / Q000473:pathology; D003105:Colombia / Q000453:epidemiology; D051180:Desmoplakins / Q000235:genetics / Q000378:metabolism*; D019353:Endemic Diseases; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000235:genetics / Q000378:metabolism; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis / Q000276:immunology / Q000378:metabolism; D007150:Immunohistochemistry; D008297:Male; D008628:Mercury / Q000009:adverse effects; D016253:Microscopy, Immunoelectron; D008875:Middle Aged; D004676:Myelin Basic Protein / Q000235:genetics / Q000378:metabolism; D009900:Optic Nerve / Q000187:drug effects / Q000276:immunology* / Q000473:pathology; D010141:Pacinian Corpuscles / Q000187:drug effects / Q000276:immunology* / Q000473:pathology; D010392:Pemphigus / Q000453:epidemiology / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D011187:Posture; D012867:Skin / Q000187:drug effects / Q000276:immunology* / Q000378:metabolism
chemical_list: D000941:Antigens; D001323:Autoantibodies; D018959:CD57 Antigens; D051180:Desmoplakins; D005904:Glial Fibrillary Acidic Protein; D007136:Immunoglobulins; D004676:Myelin Basic Protein; D008628:Mercury