Juvenile dermatitis herpetiformis

This code represents a rare, chronic, autoimmune blistering skin condition primarily affecting children. It is characterized by intensely itchy papules and vesicles, often symmetrically distributed, and is strongly associated with gluten-sensitive enteropathy (celiac disease).

Apply this code when documentation confirms a diagnosis of dermatitis herpetiformis specifically in a pediatric patient. This diagnosis is typically supported by clinical presentation, skin biopsy showing granular IgA deposits in the dermal papillae, and often serological testing for celiac disease markers.

• Ensure the patient's age is clearly documented as juvenile or pediatric to support this specific code.
• Consider co-coding for celiac disease (e.g., 579.0) if diagnosed, as there is a strong association.
• Differentiate from other blistering disorders like bullous pemphigoid or linear IgA bullous dermatosis, which have distinct pathological findings and different age predilections.