Brazilian pemphigus [fogo selvagem]

This code represents an autoimmune blistering skin disease, specifically a localized form of pemphigus foliaceus endemic to certain regions of South America, particularly Brazil. It is characterized by superficial flaccid blisters and erosions, primarily affecting the skin, often sparing mucous membranes. The condition is mediated by autoantibodies targeting desmoglein 1, leading to acantholysis in the superficial epidermis.

Use this code when documentation explicitly states a diagnosis of Brazilian pemphigus or "fogo selvagem." This diagnosis is typically made based on clinical presentation, histological findings (superficial acantholysis), and direct immunofluorescence demonstrating intercellular IgG and C3 deposition in the epidermis. It is crucial to differentiate this specific form of pemphigus from other autoimmune blistering diseases.

• Ensure the documentation clearly specifies "Brazilian pemphigus" or "fogo selvagem" to avoid confusion with other pemphigus subtypes.
• Do not use this code for other forms of pemphigus foliaceus unless the Brazilian endemic variant is explicitly stated.
• Consider additional codes for associated symptoms or complications, such as secondary infections, if documented.