Pemphigus, unspecified

This code identifies a chronic autoimmune blistering disease affecting the skin and mucous membranes, characterized by the formation of intraepidermal blisters due to autoantibodies against desmogleins. It signifies a general diagnosis of pemphigus when the specific type (e.g., vulgaris, foliaceus) is not documented.

Apply this code when the provider's documentation clearly states "pemphigus" but does not specify the particular variant. This is appropriate for initial encounters or when diagnostic workup for specific pemphigus types is still pending or inconclusive. Supporting documentation would include physician notes indicating a diagnosis of pemphigus without further detail.

• Avoid using this code if a more specific type of pemphigus is documented; always code to the highest level of specificity.
• Review the medical record for any mention of pemphigus vulgaris, pemphigus foliaceus, or other specific forms before defaulting to unspecified.
• Consider querying the provider for clarification on the type of pemphigus if the documentation is ambiguous but suggests a specific variant.