Pemphigus erythematosus

This code represents a rare autoimmune blistering disease characterized by features of both pemphigus erythematosus and lupus erythematosus. It typically presents with chronic, superficial blistering and crusting lesions, often on the face and scalp, along with positive direct immunofluorescence for IgG and C3 at the dermoepidermal junction.

Use this code when documentation clearly indicates a diagnosis of pemphigus erythematosus, a specific subtype of pemphigus. This diagnosis is usually confirmed by clinical presentation, histopathology showing acantholysis, and immunofluorescence studies demonstrating IgG and C3 deposition.

• Differentiate from other pemphigus subtypes (e.g., pemphigus vulgaris, pemphigus foliaceus) based on specific clinical and immunopathological findings.
• Ensure documentation specifies "erythematosus" to avoid miscoding to a more general pemphigus code.
• Consider co-occurring autoimmune conditions, especially lupus erythematosus, and code them separately if present and documented.