Clinical characteristics and treatment of Melkersson-Rosenthal syndrome--overview of six patients/ Klinicka prezentacija i tretman kod melkerson-rozentalovog sindroma--pregled sest pacijenata

Citation metadata

Authors: Sanvila Raskovic, Jasna Bolpacic, Aleksandra Peric Popadic, Zikica Jovicic, Rada Miskovic and Mirjana Bogic
Date: November-December 2015
From: Medicinski Pregled(Vol. 68, Issue 11-12)
Publisher: Drustvo Lekara Vojvodine
Document Type: Case study
Length: 2,111 words

Main content

Abstract :

Introduction. Melkersson-Rosenthal syndrome is a rare disease of unknown etiology. Histopathologically, it presents as granulomatous cheilitis. From laboratory aspect, it is a nonspecific, differential diagnostically and therapeutically complex condition. Case Report. This is a report of six cases treated at the Department of Allergology and Immunology of the Clinical Center of Serbia, who had presented with the referral diagnosis of recurring or persistent lip edema, and who were diagnosed with Melkersson-Rosenthal syndrome upon detailed evaluation. Three patients had complete triad of symptoms, two had the oligosymptomatic form and one manifested the monosymptomatic form of the disease. Histopathological findings of the oral mucosa specimens verified the presence of non-necrotic epithelioid granulomas in all patients. The patients were treated with the H1 and H2 antihistamines, corticosteroids, followed by anabolic drugs and antibiotics, resulting in transient and unfavorable effects. Conclusion. In differential diagnosis, Melkersson-Rosenthal syndrome diagnosis primarily refers to conditions of angioneurotic edema and hereditary angioedema, as well as granulomatous diseases such as sarcoidosis, tuberculosis and Chron's disease. It is necessary to follow-up these patients in view of monitoring the effects of the therapy and possible development of systemic granulomatous diseases. Key words: Melkersson-Rosenthal Syndrome; Morphological and Microscopic Findings; Signs and Symptoms; Diagnosis; Treatment Outcome; Lip; Edema; Cheilitis; Tongue, Fissured; Facial Paralysis; Angioedema Uvod. Melkerson-Rozentalov sindrom je retko oboljenje, nepoznate etiologije. Patohistoloski se prezentuje kao granulomatozni heilitis. Laboratorijski je nespecifican, diferencijalno-dijagnosticki i terapijski kompleksan. Lecenje ovih bolesnika je kompleksno i cesto zahteva upotrebu razlicitih terapijskih modaliteta. Prikaz slucaja. Prikazano je sest bolesnika koji su ispitivani na Klinici za alergologiju i imunologiju Klinickog centra Srbije, pod uputnom dijagnozom angioedema usana, a kod kojih je nakon detaljne evaluacije postavljena dijagnoza Melkerson-Rozentalov sindroma. Tri bolesnika su imali kompletan trijas simptoma, dva oligosimptomatsku i jedan monosimptomatsku formu. Kod svih pacijenata patohistoloski nalaz biopsije mukoze usana potvrdio je postojanje nenekroticnih epiteloidnih granuloma. Pacijenti su leceni H1 i H2 antihistaminicima, kortikosteroidima, uz antibiotike i anabolicke lekove, sa promenljivim uspehom. Zakljucak. Dijagnoza Melkerson-Rozentalov sindrom se diferencijalno-dijagnosticki odnosi pre svega na stanja rekuretnog i hereditarnog angioedema, kao i granulomatozne bolesti kao sto su sarkoidoza, tuberkuloza i Kronova bolest. Neophodno je pracenje ovih bolesnika zbog efekta primenjene terapije i mogucnosti razvoja sistemskih granulomatoznih bolesti. Kljucne reci: Melkersson-Rosenthal Sindrom; Morfoloski i mikroskopski nalazi; Znaci i simptomi; Dijagnoza; Ishod lecenja; Usna; Edem; Heilitis; Fisure jezika; Facijalna paraliza; Angioedem

Source Citation

Source Citation
Raskovic, Sanvila, et al. "Clinical characteristics and treatment of Melkersson-Rosenthal syndrome--overview of six patients/ Klinicka prezentacija i tretman kod melkerson-rozentalovog sindroma--pregled sest pacijenata." Medicinski Pregled, vol. 68, no. 11-12, Nov.-Dec. 2015, pp. 401+. Accessed 1 Dec. 2021.
  

Gale Document Number: GALE|A442779818