Hereditary angioedema type 2

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Hereditary angioedema type 2

ORPHA:100051Etiological subtype

Also called HAE 2 · HAE-II · Hereditary angioneurotic edema type 2

What it is

Hereditary angioedema type 2 (HAE 2) is a form of hereditary angioedema characterized by acute edema in subcutaneous tissues, viscera and/or the upper airway.

Key facts

Age of onset
All ages
Inheritance
Autosomal dominant
Classified as
Etiological subtype

Recorded for the broader condition

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Gene

SERPING1Disease-causing germline mutation(s)

ICD-10 codes

D84.1filed under a broader ICD-10 category — shared with 15 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Cross-references

MONDO 0015054OMIM 106100UMLS C0398776

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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