Melkersson-rosenthal syndrome presenting as a misdiagnosis of angioedema

Abstract

Background: Diagnosis of angioedema can be a challenge for physicians. Melkersson-Rosenthal syndrome (MRS) – is a rare neuro-mucocutaneous disease of unknown origin, clinically recognized by recurrent angioedema of lips and face, facial palsy, and fissured tongue. Objective: Our aim was to present the case of delayed diagnosis of MRS after two decades of clinical symptoms. Methods: 57-year-old Caucasian male patient was complaining of recurrent swelling of lips lasting for few days and resistant to antihistamines. Attacks of facial angioedema without urticaria started 20 years ago, infections, allergy, autoimmune diseases, hereditary angioedema were excluded. During the period of 20 years, the patient had recurrent episodes of tingling and tension of the lips followed by swelling of the lower part of the face, sometimes provoked by labial Herpes simplex infection. The swelling of the face usually resolved after intake of oral prednisolone, later on the asymmetric face was visible between attacks. MRS was suspected as angioedema was combined with left facial nerve palsy and fissured tongue. Patient’s mother also had a fissured tongue but no angioedema history. Lower lip biopsy and histology revealed nonspecific, inflammatory changes, infiltration of immune cells and telangiectasia and no specific changes like cheilitis granulomatosa were found. Results: Facial angioedema resistant to antihistamines was one of Melkersson-Rosenthal syndrome combined with facial palsy, fissured tongue and positive family history of partial MRS. Conclusion: Angioedema is the main symptom of MRS associated with facial palsy, and fissured tongue. Patients with angioedema should be evaluated for concomitant symptoms and family history of rare syndroms such as MRS especially in treatment resistant cases.