Clinicopathological significance of Melkersson-Rosenthal syndrome

Bohra, Shruti; Kariya, Pratik B; Bargale, Seema; Kiran, Shital

doi:10.1136/bcr-2015-210138

Cited by 10 publications

(22 citation statements)

References 19 publications

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“…This "limited inflammation" manifests clinically as the edema described above, which remits in no longer than 5 days with no residual damage. Persistent local cutaneous edema, such as the facial swelling that is characteristic of Melkersson-Rosenthal syndrome, should not be considered angioedema [2].…”

Section: Introductionmentioning

confidence: 99%

Novelties in the Diagnosis and Treatment of Angioedema

Cicardi¹,

Suffritti²,

Perego³

et al. 2016

J Investig Allergol Clin Immunol

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Angioedema is defined as local, noninflammatory, self-limiting edema that is circumscribed owing to increased leakage of plasma from the capillaries located in the deep layers of the skin and the mucosae. Two mediators, histamine and bradykinin, account for most cases of angioedema. Angioedema can occur with wheals as a manifestation of urticaria, and this form is frequently allergic. In the present review, we discuss nonallergic angioedema without wheals, which can be divided into 3 acquired and 4 hereditary forms. Histamine is the mediator in acquired angioedema of unknown etiology (idiopathic histaminergic acquired angioedema), whereas in other forms the main mediator is bradykinin. Angioedema can be caused by C1-inhibitor deficiency (C1-INH-hereditary angioedema and C1-INH-acquired angioedema), mutations in coagulation factor XII (FXII-hereditary angioedema), and treatment with angiotensin-converting enzyme inhibitors (ACEI-acquired angioedema). Etiology remains unclear in acquired angioedema (idiopathic nonhistaminergic acquired angioedema) and in 1 type of hereditary angioedema (hereditary angioedema of unknown origin). Several treatments are licensed for hereditary C1-INH deficiency. Plasma-derived and recombinant C1-INHs, the bradykinin receptor blocker icatibant, and the plasma kallikrein inhibitor ecallantide have been approved for on-demand treatment to reverse angioedema symptoms. Attenuated androgen and plasma-derived C1-INH are approved for prophylaxis. Key words: Angioedema. Bradykinin. Histamine. C1 inhibitor. Coagulation factor XII. Angiotensin-converting enzyme inhibitors. Urticaria. ResumenAngioedema se define como un edema local, autolimitado, no-inflamatorio. Se trata de un edema circunscrito debido a la trasvasación de plasma de los capilares localizados en los sustratos profundos de la piel y de las mucosas. En la mayoría de los casos están implicados dos mediadores, la histamina y la serotonina. Puede manifestarse en forma de habones como en la urticaria de origen alérgico. El angioedema de origen no alérgico es el motivo de esta revisión. Se puede presentar bajo 3 formas adquiridas y 4 formas hereditarias. La histamina es el mediador implicado en el angioedema adquirido de etiología desconocida (angioedema adquirido idiopático histaminérgico). En las otras formas se sospecha que es la serotonina el mediador principal. La etiología del angioedema puede ser identificado en 4 tipos: una deficiencia de C1-inhibidor (C1-INH-angioedema hereditario y C1-INH-angioedema adquirido), mutaciones en el factor XII de coagulación (FXII-angioedema hereditario), tratamiento con inhibidores del enzima convertidor de la angiotensina (ACEi-angioedema adquirido). En uno de los adquiridos (angioedema adquirido idiopático no histaminérgico) y en el hereditario de origen desconocido, no se ha identificado todavía su etiología. Varios tratamientos están aprobados para revertir los síntomas clínicos y se aplican en la deficiencia de angioedema hereditario por déficit de C1-INH: Derivados de plasma y C1-IN...

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Section: Introductionmentioning

confidence: 99%

Novelties in the Diagnosis and Treatment of Angioedema

Cicardi¹,

Suffritti²,

Perego³

et al. 2016

J Investig Allergol Clin Immunol

View full text Add to dashboard Cite

show abstract

“…Consiste en la asociación, no necesariamente simultánea aunque sí con frecuencia recidivante, de parálisis facial recidivante, lengua fisurada y edematización orofacial, apareciendo esta triada típica en tan solo un tercio de los pacientes (1,2) lo que dificulta el diagnóstico. Pueden aparecer otras alteraciones neurológicas, como trastornos del sentido del gusto, neuralgia del trigémino, migraña o disartria (2,3). Su incidencia se estima en torno al 0.08%, habiéndose descrito algunos casos de agregación familiar (3).…”

Section: Estimado Editor;unclassified

“…Dado el desconocimiento etiopatogénico de este cuadro clínico, su tratamiento es sintomático, recomendándose tratamiento con AINEs y antihistamínicos en casos leves, reservándose el tratamiento con corticoides para los episodios de parálisis facial (2); en algunos casos refractarios a tratamiento médico puede ser necesario plantear la descompresión quirúrgica del nervio facial (2,3).…”

Section: Estimado Editor;unclassified

60 year old woman with lip edema and fissured tongue

Berná¹,

Baena²,

Díaz³

2016

Actual. Med.

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“…Melkersson-Rosenthal syndrome is a rare granulomatous disease that classically consists of a triad of recurrent orofacial oedema, facial nerve palsy and fissured tongue 1–3. It usually presents in a monosymptomatic or oligosymptomatic form and oedema of the lips is the most common monosymptomatic presentation, also termed Meischer’s syndrome or cheilitis granulomatosa.…”

Section: Introductionmentioning

confidence: 99%

“…Several comorbidities have been reported in association with this disease. However, the underlying aetiology and pathogenesis remain unknown 1 4…”

Section: Introductionmentioning

confidence: 99%

Melkersson-Rosenthal syndrome with coeliac and allergic diseases

Martins¹,

Azenha

Almeida

et al. 2019

BMJ Case Rep

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A 45-year-old man presented with a 10-year history of relapsing oedema of the lips. Moreover, he exhibited recurrent facial nerve palsy since the age of 10 years, coeliac disease since the age of 12 years, atopic eczema, allergic rhinitis and asthma. Physical examination revealed lip swelling and lingua plicata. Thus, he presented the classic triad of Melkersson-Rosenthal syndrome which includes recurrent orofacial oedema, facial nerve palsy and fissured tongue. A lip biopsy confirmed our clinical diagnosis.This case is particularly rare, as the classic triad is seen only in a minority of the cases. Moreover, allergic and coeliac diseases were observed concomitantly. This paper illustrates a potential pathophysiological interconnection between these pathologies in which interferon gamma could play a key role. To our knowledge, this is the first case report in which Melkersson-Rosenthal syndrome has been observed concurrently with coeliac disease.

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Clinicopathological significance of Melkersson-Rosenthal syndrome

Cited by 10 publications

References 19 publications

Novelties in the Diagnosis and Treatment of Angioedema

Novelties in the Diagnosis and Treatment of Angioedema

60 year old woman with lip edema and fissured tongue

Melkersson-Rosenthal syndrome with coeliac and allergic diseases

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