Pemphigus

This code represents pemphigus, a rare group of autoimmune blistering diseases affecting the skin and mucous membranes. It is characterized by the formation of intraepidermal blisters due to autoantibodies targeting desmogleins, proteins responsible for cell-to-cell adhesion.

Use this code for patients diagnosed with any form of pemphigus, including pemphigus vulgaris, pemphigus foliaceus, or paraneoplastic pemphigus. Documentation should clearly state a diagnosis of pemphigus, often supported by biopsy results showing acantholysis and direct immunofluorescence demonstrating intercellular IgG deposits.

• Differentiate from bullous pemphigoid (694.5), which involves subepidermal blistering.
• Specify the type of pemphigus when possible, though this code covers all forms.
• Consider additional codes for complications such as secondary infections (e.g., cellulitis) or systemic manifestations.