Erythema marginatum preceding hereditary angioedema

Starr, John C.; Brasher, George W.

doi:10.1016/0091-6749(74)90119-5

Cited by 38 publications

(13 citation statements)

References 8 publications

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“…Urticaria is not a feature of C1 INH deficiency. However, prodromal erythema has been reported in up to 25% of patients which may be mistaken for urticaria [16,17].…”

Section: Clinicalmentioning

confidence: 99%

C1 inhibitor deficiency: consensus document

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Abinun

et al. 2005

Clinical and Experimental Immunology

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SummaryWe present a consensus document on the diagnosis and management of C1 inhibitor deficiency, a syndrome characterized clinically by recurrent episodes of angio-oedema. In hereditary angio-oedema, a rare autosomal dominant condition, C1 inhibitor function is reduced due to impaired transcription or production of non-functional protein. The diagnosis is confirmed by the presence of a low serum C4 and absent or greatly reduced C1 inhibitor level or function. The condition can cause fatal laryngeal oedema and features indistinguishable from gastrointestinal tract obstruction. Attacks can be precipitated by trauma, infection and other stimulants. Treatment is graded according to response and the clinical site of swelling. Acute treatment for severe attack is by infusion of C1 inhibitor concentrate and for minor attack attenuated androgens and/or tranexamic acid. Prophylactic treatment is by attenuated androgens and/or tranexamic acid. There are a number of new products in trial, including genetically engineered C1 esterase inhibitor, kallikrein inhibitor and bradykinin B2 receptor antagonist. Individual sections provide special advice with respect to diagnosis, management (prophylaxis and emergency care), special situations (childhood, pregnancy, contraception, travel and dental care) and service specification.

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“…Urticaria is not a feature of C1 INH deficiency. However, prodromal erythema has been reported in up to 25% of patients which may be mistaken for urticaria [16,17].…”

Section: Clinicalmentioning

confidence: 99%

C1 inhibitor deficiency: consensus document

Gompels

Lock

Abinun

et al. 2005

Clinical and Experimental Immunology

379

460

View full text Add to dashboard Cite

show abstract

“…23 This is perhaps unsurprising, given the similarity in clinical picture between HAE and other forms of AE. For example, the prodromal erythema that affects up to 25% of patients with HAE 19 can be mistakenly identified as urticaria.…”

Section: Differential Diagnosismentioning

confidence: 96%

“…A prodrome of erythema marginatum (Fig 3) has been reported in up to 25% of patients 19 and may be confused with urticaria.…”

Section: Clinical Aspects Of Haementioning

confidence: 98%

Hereditary angioedema: Diagnosis and management–a perspective for the dermatologist

Parish

2011

Journal of the American Academy of Dermatology

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“…1 Some patients experience symptoms, such as pruritus, nausea, fatigue, or malaise, that precede the onset of an angioedematous attack 2,3 and that may enable the patient to predict an impending attack and trigger early intervention. One commonly reported prodromal symptom is erythema marginatum, a nonpitting, nonpruritic, serpiginous rash first described by Osler in 1888, 4 which can manifest hours to days before the onset of a C1-INH-HAE attack [5][6][7] or may even develop independently of the angioedema. Erythema marginatum occurs in ∼42% to 58% of pediatric patients with C1-INH-HAE, but is nevertheless often mistaken for urticaria.…”

mentioning

confidence: 99%

Erythema Marginatum as an Early Symptom of Hereditary Angioedema: Case Report of 2 Newborns

Martinez‐Saguer¹,

Farkas

2016

Pediatrics

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Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare genetic disease that causes recurrent swelling attacks that may affect various body tissues. Angioedematous attacks can be fatal in the case of upper airway edema and are often preceded by prodromal symptoms like erythema marginatum. Initial symptoms usually occur in the first decade of life. We report on manifestation of profound and recurrent erythema marginatum in 2 newborns. In both cases, prodromal symptoms could help determine the diagnosis of C1-INH-HAE such that, at a later time, angioedematous attacks could be treated promptly and effectively. Awareness of C1-INH-HAE is low among physicians and even lower among the general public. This report aims at raising the level of awareness and shows that initial symptoms of the potentially life-threatening condition can manifest in newborns and that erythema marginatum can even be present at birth. Recognition of early symptoms and timely diagnosis of the disease along with adequate education of the pediatrician and parents are a prerequisite for prompt and effective treatment of attacks and the successful management of the disease.

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Erythema marginatum preceding hereditary angioedema

Cited by 38 publications

References 8 publications

C1 inhibitor deficiency: consensus document

C1 inhibitor deficiency: consensus document

Hereditary angioedema: Diagnosis and management–a perspective for the dermatologist

Erythema Marginatum as an Early Symptom of Hereditary Angioedema: Case Report of 2 Newborns

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