Macroheilitis as a manifestation of Melkersson – Rosenthal syndrome

Melkersson – Rosenthal syndrome (SMR) is a disease manifested by a triad of symptoms: swelling of the lips and/or face, paralysis of the facial nerve, scrotal tongue. In clinical practice, the monosymptomatic variant of SMR is more common. The disease can develop at any age, but most cases occur at 25–40 years with a 2-fold predominance of women among patients. In children, SMR is more often observed at the age of 7 to 12 years, and the smallest child is described at the age of 22 months. The causes of the Melkersson – Rosenthal syndrome have not been definitively clarified. There are many hypotheses about the pathogenesis of SMR, the main of which are allergic, angioedema, infectious and genetic, immunological dysfunction. There are various historically established terms: Mischer’s granulomatous cheilitis, orofacial granulomatosis, SMR, which are regularly used as synonyms in the available literature. To date, SMR remains a diagnosis of exclusion, which is accompanied by the establishment of controversial diagnoses in practical healthcare. In most cases, the treatment of SMR is ineffective. Mainly systemic glucocorticosteroids, antibiotics, antihistamines, immunosuppressive drugs are used, upon discontinuation of which the disease often recurs. In this publication, we present two clinical cases of SMR. The interest of the first case lies in the presence of the entire group of clinical symptoms and the achievement of a positive treatment result. The second case is characterized by the achievement of long-term remission as a result of combination therapy with betamethasone, azithromycin and ketotifen. An overview of successful treatment options is also presented.

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