Arquivos de Asma, Alergia e Imunologia
https://aaai-asbai.org.br/article/doi/10.5935/2526-5393.20190047
Arquivos de Asma, Alergia e Imunologia
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Síndrome de Melkersson-Rosenthal

Melkersson-Rosenthal syndrome

Adriana Pereira de Lira Marques; Paulo Eduardo Silva Belluco

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Resumo

A síndrome de Melkersson-Rosenthal é uma doença rara que pode se apresentar como uma tríade clássica de edema orofacial, paralisia facial e língua fissurada ou, mais frequentemente, com características oligo/monossintomáticas. Relatamos um caso que aportou a um alergista para o diagnóstico de um angioedema, e que na avaliação se configurou como a síndrome completa. Diagnósticos diferenciais com angioedema alérgico, hereditário, idiopático e com outras patologias devem ser considerados. Apesar de o diagnóstico ser clínico, a biópsia cutânea foi relevante. Objetivamos alertar o especialista que se depara com angioedema crônico recorrente sobre essa patologia.

Palavras-chave

Síndrome de Melkersson-Rosenthal, angioedema, paralisia facial, língua fissurada.

Abstract

Melkersson-Rosenthal syndrome is a rare disease that may present as the classic triad of orofacial edema, facial paralysis, and fissured tongue or, more often, with oligosymptomatic/ monosymptomatic features. We report a case referred to an allergist for the diagnosis of angioedema that was identified as a complete syndrome during evaluation. Differential diagnoses with allergic, hereditary, and idiopathic angioedema and with other diseases should be considered. Although the diagnosis is clinical, skin biopsy is relevant. Our goal is to alert specialists who deal with chronic recurrent angioedema to the possibility of this syndrome.

Keywords

Melkersson-Rosenthal syndrome, angioedema, facial paralysis, fissured tongue.

References

1. Cancian M, Giovannini S, Angelini A, Fedrigo M, Bendo R, Senter R, et al. Melkersson-Rosenthal syndrome: a case report of a rare disease with overlapping features. Allergy, Asthma Clin Immun. 2019;15:1-5.

2. Patterson JW. Weedon’s Skin Pathology. 4ª ed. Londres: Elsevier; 2016.

3. Lin TY, Chiang CH, Cheng PS. Melkersson-Rosenthal syndrome. J Formosan Med Ass. 2016;115:583-4.

4. Mansour M, Mahmoud MB, A Kacem, Zaouali J, Mrissa R. Melkersson-Rosenthal syndrome: About a Tunisian family and review of the literature. Clinical Neurology and Neurosurgery. 2019;185:105457.

5. James W, Elston D, Treat R, Rosenbach M, Neuhaus I. Andrew’s Diseases of the Skin. 13ª ed. Londres: Elsevier; 2019.

6. Bakshi SS. Melkersson-Rosenthal syndrome. J Allergy Clin Immunol Pract. 2017;5(2):471‑2.

7. Moraes OC, Tinoco IF, Carvalho JL, et al. Queilite de Miescher monossitomática da síndrome de Melkersson-Rosenthal: Relato de caso. XLIII Congresso Brasileiro de Alergia e Imunologia. 2016; PO 267.

8. Haaramo A, Kolho KL, Pitkaranta A. A 30-year follow-up study of patients with Melkersson-Rosenthal syndrome shows an association to inflammatory bowel disease. Ann Med. 2019;51(2):149-55.


Submitted date:
09/10/2019

Accepted date:
09/16/2019

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