Dermatitis herpetiformis

This code represents a chronic, intensely itchy blistering skin condition characterized by symmetrically distributed papules, vesicles, and bullae, primarily affecting extensor surfaces. It is an autoimmune disorder strongly associated with celiac disease, though not all patients experience gastrointestinal symptoms.

Apply this code when documentation confirms a diagnosis of dermatitis herpetiformis in an adult patient. This diagnosis is typically supported by characteristic skin lesions, severe pruritus, and often confirmed by direct immunofluorescence showing granular IgA deposits in the dermal papillae.

• Do not use this code for pediatric or geriatric patients; refer to the Excludes1 notes for age-specific codes.
• Always review documentation for mention of associated celiac disease, which would require an additional code.
• Ensure the provider's documentation clearly distinguishes this condition from other blistering disorders like bullous pemphigoid or linear IgA bullous dermatosis.