Melkersson–Rosenthal syndrome

Melkersson–Rosenthal syndrome QJM: An International Journal of Medicine, 2018, 199 doi: 10.1093/qjmed/hcx210 Advance Access Publication Date: 27 October 2017 Clinical picture CLINICAL PI CTURE A 41-year-old woman presented to our clinic with a painful For most therapeutic options, the clinical results achieved are swollen upper lip (Figure 1a) and a fissured tongue (Figure 1b). unsatisfactory. For the persistent swollen lips, a surgical or radi- She reported about progressive symptoms for more than 6 ation therapy has been discussed as ultimo ratio. months. Furthermore, she had at least three episodes of facial paralysis on her left side. Other comorbidities could be excluded Photographs and text from: J.M. Placke, M. Moelleken and by different physicians. A biopsy from her upper lip showed J. Dissemond, Department of Dermatology, Venerology and histological a granulomatous cheilitis, so that we diagnosed a Allergology, University School of Medicine Essen-Duisburg, Melkersson–Rosenthal syndrome (MRS, MIM 155900). Hufelandstrasse 55, 45122 Essen, Germany. email: joachim.dis- The MRS is a rare multidisciplinary relevant disease with an in- semond@uk-essen.de cidenceof1:1250and an unknown pathogenesis. Usually ear, nose and throat specialists, neurologists and dermatologists are involved in finding of the diagnosis. The disease is characterized by the clin- ical symptom triad of orofacial oedema, lingua plicata and recur- Conflict of interest: None declared. rent usually unilateral facial paralysis. Mostly the symptoms occur not at the same time. Therefore, it often takes many months to References years from the first symptom to the correct clinical diagnosis. 1. Talabi OA. Melkerssons-Rosenthal syndrome: a case report Recurrent episodes of erysipelas, herpes simplex or furuncles of and review of the literature. Niger J Clin Pract 2011; 14:477–8. the upper lip are common misdiagnoses. The patients often suffer 2. Critchlow WA, Chang D. Cheilitis granulomatosa: a review. from the chronic and spasmodic progress of the disease. The histo- Head Neck Pathol 2014; 8:209–13. pathology typically demonstrates a non-caseating granulomatous 3. Xu XG, Guan LP, Lv Y, Wan YS, Wu Y, Qi RQ, et al.Exome cheilitis. In a Chinese family, a gene mutation in fatty acid trans- sequencing identifies FATP1 mutation in Melkersson-Rosenthal port protein 1 related to anMRS hadbeendescribed. syndrome. J Eur Acad Dermatol Venereol 2017; 31:230–2. Therapeutically an external therapy with intralesional ster- 4. Camacho F, Garcı ´a-Bravo B, Carrizosa A. Treatment of oids or triamcinolone can be tried. In the case of lacking success, Miescher‘s cheilitis granulomatosa in Melkersson-Rosenthal an internal therapy with clofazimin, sulfonamides, acetylsalicyl- syndrome. J Eur Acad Dermatol Venereol 2001; 15:546–9. clic acid, chloroquine, dapsone or systemic steroids can be given. Figure 1. The MRS with (a) swelling of the upper lip and (b) lingua plicata. V C The Author 2017. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oup.com Downloaded from https://academic.oup.com/qjmed/article-abstract/111/3/199/4568571 by Ed 'DeepDyve' Gillespie user on 16 March 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png QJM: An International Journal of Medicine Oxford University Press

Melkersson–Rosenthal syndrome

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Oxford University Press
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© The Author 2017. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oup.com
ISSN
1460-2725
eISSN
1460-2393
D.O.I.
10.1093/qjmed/hcx210
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Abstract

QJM: An International Journal of Medicine, 2018, 199 doi: 10.1093/qjmed/hcx210 Advance Access Publication Date: 27 October 2017 Clinical picture CLINICAL PI CTURE A 41-year-old woman presented to our clinic with a painful For most therapeutic options, the clinical results achieved are swollen upper lip (Figure 1a) and a fissured tongue (Figure 1b). unsatisfactory. For the persistent swollen lips, a surgical or radi- She reported about progressive symptoms for more than 6 ation therapy has been discussed as ultimo ratio. months. Furthermore, she had at least three episodes of facial paralysis on her left side. Other comorbidities could be excluded Photographs and text from: J.M. Placke, M. Moelleken and by different physicians. A biopsy from her upper lip showed J. Dissemond, Department of Dermatology, Venerology and histological a granulomatous cheilitis, so that we diagnosed a Allergology, University School of Medicine Essen-Duisburg, Melkersson–Rosenthal syndrome (MRS, MIM 155900). Hufelandstrasse 55, 45122 Essen, Germany. email: joachim.dis- The MRS is a rare multidisciplinary relevant disease with an in- semond@uk-essen.de cidenceof1:1250and an unknown pathogenesis. Usually ear, nose and throat specialists, neurologists and dermatologists are involved in finding of the diagnosis. The disease is characterized by the clin- ical symptom triad of orofacial oedema, lingua plicata and recur- Conflict of interest: None declared. rent usually unilateral facial paralysis. Mostly the symptoms occur not at the same time. Therefore, it often takes many months to References years from the first symptom to the correct clinical diagnosis. 1. Talabi OA. Melkerssons-Rosenthal syndrome: a case report Recurrent episodes of erysipelas, herpes simplex or furuncles of and review of the literature. Niger J Clin Pract 2011; 14:477–8. the upper lip are common misdiagnoses. The patients often suffer 2. Critchlow WA, Chang D. Cheilitis granulomatosa: a review. from the chronic and spasmodic progress of the disease. The histo- Head Neck Pathol 2014; 8:209–13. pathology typically demonstrates a non-caseating granulomatous 3. Xu XG, Guan LP, Lv Y, Wan YS, Wu Y, Qi RQ, et al.Exome cheilitis. In a Chinese family, a gene mutation in fatty acid trans- sequencing identifies FATP1 mutation in Melkersson-Rosenthal port protein 1 related to anMRS hadbeendescribed. syndrome. J Eur Acad Dermatol Venereol 2017; 31:230–2. Therapeutically an external therapy with intralesional ster- 4. Camacho F, Garcı ´a-Bravo B, Carrizosa A. Treatment of oids or triamcinolone can be tried. In the case of lacking success, Miescher‘s cheilitis granulomatosa in Melkersson-Rosenthal an internal therapy with clofazimin, sulfonamides, acetylsalicyl- syndrome. J Eur Acad Dermatol Venereol 2001; 15:546–9. clic acid, chloroquine, dapsone or systemic steroids can be given. Figure 1. The MRS with (a) swelling of the upper lip and (b) lingua plicata. V C The Author 2017. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oup.com Downloaded from https://academic.oup.com/qjmed/article-abstract/111/3/199/4568571 by Ed 'DeepDyve' Gillespie user on 16 March 2018

Journal

QJM: An International Journal of MedicineOxford University Press

Published: Mar 1, 2018

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