Dermatitis herpetiformis

This code represents dermatitis herpetiformis, a chronic, intensely itchy, blistering skin condition characterized by symmetrically distributed papules and vesicles. It is an autoimmune disorder strongly associated with celiac disease, where gluten ingestion triggers the immune response.

Use this code for patients diagnosed with dermatitis herpetiformis based on characteristic skin lesions, severe pruritus, and often confirmed by skin biopsy showing granular IgA deposits in the dermal papillae. Documentation should clearly indicate the diagnosis of dermatitis herpetiformis.

• Ensure documentation differentiates from other blistering skin conditions like bullous pemphigoid or pemphigus.
• Consider co-coding for celiac disease (e.g., 579.0) if diagnosed, as there is a strong association.
• Look for specific mention of IgA deposits on biopsy to support the diagnosis.