EB44

ICD-11 MMS 2026-01

Dermatitis herpetiformis

Description

Dermatitis herpetiformis is an immunobullous skin characterised by recurrent eruptions of intensely itchy papules, vesicles or bullae, which are typically grouped symmetrically on the extensor surfaces of the limbs and on the buttocks and back. The primary lesions are frequently obscured by excoriation. An incompletely understood abnormal response to dietary gluten provokes the formation of autoantibodies to tissue and epidermal transglutaminases and granular deposition of IgA in dermal papillae. The disease is strongly associated with gluten-sensitive enteropathy, which can range from mild jejunal epithelial inflammation to total villous atrophy (coeliac disease).

Index terms

  • Dermatitis herpetiformis
  • DH - [dermatitis herpetiformis]
  • Duhring–Brocq disease

Exclusions from above levels

  • 1B72.0 – Bullous impetigo of the napkin area
  • 1F23.12 – Napkin candidosis
  • EH71 – Dermatoses precipitated by drug therapy
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  • EH76 – Dermatoses associated with specific classes of medication

Sibling codes

  • EB40 – Pemphigus
  • EB41 – Pemphigoid
  • EB42 – Linear IgA bullous dermatosis
  • EB43 – Epidermolysis bullosa acquisita
  • EB4Y – Other specified immunobullous disorder

Foundation entity

Dermatitis herpetiformis – http://id.who.int/icd/entity/286313127

Part of: 14: Diseases of the skin