fogo selvagem
[Portuguese fogo selvagem, wild fire]
ABBR: FS
An autoimmune disease that causes blistering of the skin of the head, neck or trunk, esp. when the skin is rubbed (Nikolsky sign).
Incidence: The disease is typically found in Brazil or Colombia. It has been associated with chronic or recurrent exposure to black flies.
Symptoms and Signs: FS often causes an unpleasant burning sensation (dysesthesia) on the skin, from which the popular name of the disease is derived.
Diagnosis: Skin biopsies reveal blistering beneath the corneal layer of the skin with acantholysis (detachment of keratinocytes). Immunoglobulin G (IgG) autoantibodies against intracellular adhesion molecules (anti-desmoglein 1-(Dsg1) are pathogenic and are found in the serum of affected patients and on direct immunofluorescent staining of skin biopsies.
Treatment: Imiquimod (a topical immune response modifier), immune-suppressing drugs, and monoclonal antibody therapies have been used to treat all forms of pemphigus foliaceus, which includes FS.
Patient Care: Blisters should be kept clean and dry and should not be disturbed (rubbed, scratched, or popped). Affected skin should be protected from abrasion against neighboring body parts or clothing, and kept out of direct sunlight. Professional health care should be sought when blisters become painful or obviously infected or when they form near or in the eyes, mouth, or anus. SYN: endemic pemphigus foliaceus..
