DA01.21

ICD-11 MMS 2026-01

Orofacial granulomatosis

Description

Orofacial granulomatosis (OFG) is an uncommon clinicopathological entity characterised by persistent and/or recurrent lymphoedema and fissuring of the lips and oral mucosa (the latter resulting in a "cobblestone" appearance), oral ulceration and the presence on biopsy of non-caseating granulomas. OFG may be an early manifestation of Crohn disease, sometimes presenting months or years before bowel disease. Some cases are associated with dental infections, sarcoidosis, food allergy or contact allergy. Granulomatous cheilitis is a form of OFG confined to the lips. Rarely, OFG may be associated with fissured tongue and facial palsy (Melkersson-Rosenthal syndrome).

Index terms

  • Orofacial granulomatosis

Exclusions from above levels

  • 1C1H – gangrenous stomatitis
  • DA00.0 – Cheilitis
  • DA0B – Gingival diseases
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  • DA0C.31 – Noma
  • 01 – Certain infectious or parasitic diseases
  • 02 – Neoplasms
  • 05 – Endocrine, nutritional or metabolic diseases
  • 06 – Mental, behavioural or neurodevelopmental disorders
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • DA01.20 – Giant cell granuloma, central
  • DA01.2Y – Other specified granuloma or granuloma-like lesions of oral mucosa
  • DA01.2Z – Granuloma or granuloma-like lesions of oral mucosa, unspecified

Foundation entity

Orofacial granulomatosis – http://id.who.int/icd/entity/239721777

Part of: DA01.2: Granuloma or granuloma-like lesions of oral mucosa › DA01: Disorders of oral mucosa › 13: Diseases of the digestive system