Pemphigus vegetans

Pemphigus vegetans is a rare, chronic autoimmune blistering disease characterized by flaccid bullae that rupture to form verrucous, vegetative plaques, often in intertriginous areas. It is a variant of pemphigus vulgaris, distinguished by its hypertrophic, papillomatous lesions. The condition results from autoantibodies targeting desmoglein 3, leading to acantholysis within the epidermis.

This code is used when documentation specifically identifies pemphigus vegetans. It applies to patients presenting with the characteristic vegetative lesions following initial bullae formation. Confirmation by biopsy showing acantholysis and direct immunofluorescence demonstrating intercellular IgG and C3 deposition is typically present.

• Differentiate from pemphigus vulgaris (L10.0) if vegetative lesions are not explicitly documented.
• Do not confuse with other bullous disorders like bullous pemphigoid (L12.0) or dermatitis herpetiformis (L10.5).
• Code any associated complications, such as secondary infections, separately.