Hereditary Angioedema

ICD-10: D84.1MeSH: D054179ORPHA: 91385

Overview

Hereditary angioedema is an autosomal dominant disorder caused by deficiency or dysfunction of C1 esterase inhibitor, leading to recurrent episodes of severe swelling affecting the skin, gastrointestinal tract, and upper airways. Three types are recognized: Type I (85% of cases) with low C1-INH levels, Type II with dysfunctional C1-INH, and Type III with normal C1-INH. The estimated prevalence is 1 in 50,000 individuals worldwide.

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Classification & Codes

ICD-10 Code

D84.1

MeSH Code

D054179

Orphanet Code

ORPHA:91385
Hereditary Angioedema
ICD-10D84.1
MeSHD054179
OrphanetORPHA:91385
Treatments0 drug(s)
Statuspublished
Factual Authority
Last Updated3/21/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
Hereditary Angioedema | OrphanDrug