Pseudoangioedema

Abstract

Angioedema is a rapid, localized and temporary subcutaneous edema, which targets the lips, eyelids, gastrointestinal and respiratory mucosa resulting in abdominal pain, asthma and even serious life-threatening conditions like airway obstruction. There are several other disorders such as allergic contact dermatitis, drug rash with eosinophilia and systemic symptoms (DRESS), superior vena cava syndrome (SVCS), orofacial granulomatosis and so on, which manifest with subcutaneous swelling and masquerade as angioedema and are known as 'pseudoangioedema' in the literature. Knowledge of pseudoangioedema for healthcare professionals is crucial to avoid potentially serious results of misdiagnosis such as further investigations, unnecessary applications and delayed diagnosis. We aim to discuss differential diagnosis of angioedema and help physicians recognize the typical features of angioedema and its differential diagnosis in this chapter.

If not angioedema, what is it? Diagnostic approach to facial edema

“…3 Heliotrope eruption is a typical clinical feature and is defined as a periorbital erythema associated with a symmetrically distributed edema. 4 Although it may mimic angioedema due to the periorbital edema, the associated symmetrical proximal muscle weakness, fatigue, weight loss, and elevated creatine kinase levels make it distinct from angioedema. 3 Published cases support the hypothesis that pseudoangioedema may have a poor prognosis in dermatomyositis patients, requiring assessment of underlying disorders and aggressive management as its development coincides with exacerbations.…”

“…2 Angioedema without urticaria, also known as angioneurotic edema or Quincke's edema, is divided into two main categories: hereditary angioedema and acquired angioedema. 3,4 Three main types of hereditary angioedema exist: type I, type II, and type III. tion in the SERPING1 gene, which encodes C1-INH.…”

“…Current guidelines now recommend subdividing hereditary angioedema with normal C1 esterase inhibitor gene (HAE-nl-C1-INH formerly known as HAE type III) based on underlying gene mutations, such as in kininogen-1 (HAE-KNG1), plasminogen (PLG-HAE), myoferlin (MYOF-HAE), heparan sulfate-glucosamine 3-sulfotransferase 6 (HS3ST6), Hageman factor (factor XII), and angiopoietin-1 (HAE-ANGPT-1). 4,5 Similarly, different types of acquired angioedema (AAE) have been identified. The most frequent cause of acquired facial angioedema is acute spontaneous urticaria which could be idiopathic or elicited in the context of numerous triggers of different nature, including infectious diseases and insect bites.…”

“…Apart from histamine angioedema, other types of AAE include idiopathic non-histamine angioedema, angiotensin-converting enzyme inhibitor-associated acquired angioedema, C1-lnhibitor-deficient acquired angioedema, sartan induced angioedema, angioedema triggered by NSAID intake or m-TOR inhibitor induced angioedema. 4 Several conditions can cause subcutaneous edema and are often misdiagnosed as angioedema. 4 The term "pseudoangioedema" is used to describe these conditions that mimic angioedema.…”

“…4 Several conditions can cause subcutaneous edema and are often misdiagnosed as angioedema. 4 The term "pseudoangioedema" is used to describe these conditions that mimic angioedema. 1,2 Pseudoangioedema should be considered in any patient with unusual symptoms or atypical features of angioedema.…”