Article: Volume 49 Number 1 Page 57 - January 2022

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  Dental Update 496: 57-62

Oral medicine:  Hereditary Angioedema Type I Triggered by Dental Treatment: A Case Report and Review of the Literature

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Abstract: Type 1 hereditary angioedema (HAE) is a rare genetic condition characterized by recurrent episodes of oedema caused by a deficiency of C1-esterase inhibitor (C1-INH). A 29-year-old male presented to the oral medicine department at Guy’s Hospital, London, with lip swelling following crown preparation and impressions. Haematological investigations showed reduced levels of complement C4 (0.02 g/L; reference range 0.1–0.4 g/L) and C1-INH function was <31% (reference range 85–99%). Immunology confirmed the diagnosis of type 1 HAE, with a de novo mutation. This case highlights how a detailed medical history and multidisciplinary teamwork ensure the correct diagnosis and management.

Clinical relevance: To demonstrate the various dental triggers, relevant signs and symptoms, and management options for patients diagnosed with hereditary angioedema to allow for effective decision-making in a primary dental setting.

Author notes: Yashoda Jagatiya, BDS, MFDS, RCSEd, Specialty Dentist in Oral Medicine, Department of Oral Medicine, Dental Institute, The Royal London Dental Hospital, London. Sangeetha Yogarajah, BDS, MFDS, RCSEd, PGCert, ACF in Oral Medicine, Dental Institute, Guy's and St Thomas' NHS Foundation Trust, London. Luis Monteiro, DDS, MSc, PhD, Dip Oral Med, Specialist in Oral surgery. University Institute of Health Sciences (IUCS-N), Department of Medicine and Oral Surgery and Institute of Research and Advanced Training in Health Sciences and Technologies (IINFACTS), University Institute of Health Sciences (IUCS-N), Paredes, Portugal. Barbara Carey, MB BCh BAO BDS NUI BA FDS (OM) RCS (Eng) FFDRCSI (Oral Medicine), FHEA, Consultant in Oral Medicine, Department of Oral Medicine, Dental Institute, Guy’s and St Thomas’ NHS Foundation Trust, London. Rui Albuquerque, LMD, MS, DAS, PhD, PGCME, FHEA, FDS RCS(OM), Consultant in Oral Medicine Department of Oral Medicine, Dental Institute, Guy’s and St Thomas’ NHS Foundation Trust, London; Honorary Clinical Senior Lecturer, King’s College London. email: rui.albuquerque@gstt.nhs.uk

Objective: The reader should understand the clinical implications of hereditary angioedema in a dental environment.