Granulomatous cheilitis of miescher and lingua plicata as an oligosymptomatic form of Melkersson–Rosenthal syndrome: case report and review of literature

Abstract

Abstract. Introduction: Granulomatous cheilitis of Miescher (GHM) is a rare chronic disorder that presents with recurrent swelling of one or both lips, frequently accompanied by erythema and edema of the whole face. The association of cheilitis with fissured tongue and facial paralysis is known as Melkersson–Rosenthal syndrome. It is a condition with a characteristic manifestation, unknown etiology and difficult treatment. Clinical Case Description: We present the case of a 49-year-old woman who was admitted in our dermatology department with orofacial edema for the last 10 years and fissured tongue. No family history data on medication, food, insect allergy, atopy, asthma or associated comorbidities were reported. The diagnostic workup included clinical investigation, histopathology of skin, laboratory tests and a CT scan of the head to exclude abnormalities of n. facialis canals in reference to Melkersson–Rosenthal syndrome. The patient was treated with dexamethasone 4 mg/24 h and ceftriaxone 2 g/24 h without improvement. As a second line, dapsone therapy was initiated with gradually increasing dosage with methemoglobin levels monitoring. Discussion: There is still no effective therapy for this condition, although various symptomatic therapies targeted to avoid relapses, especially in the edematous stage, exist. The aim of the treatment is to improve the clinical symptoms and quality of life. The spontaneous vanishing of the disease is very rare but such cases has been reported.