hrvatski

Melkersson-Rosenthal sindrom udružen s oligodoncijom u 15 godišnje djevojčice – prikaz slučaja

Objective: We report a case of Melkersson- Rosenthal syndrome (MRS) associated with oligodontia in 15 years old girl. Background: MRS is a rare neuromucocutaneous disorder of uncertain etiology causing localized edema, inflammation in the face and oral cavity, fissuring of the tongue, and recurrent unilateral or alternating facial palsy. Complete clinical form is uncommon and presentation is usually monosymptomatic or oligosymptomatic. Design/Methods: Our patient is a girl at the age of 15 years with recurrent facial palsy (at the age 6, 8 and two times 15), three times left-sided and once right-sided. Initial symptom was unilateral localized non-painful tongue edema, followed by ipsilateral facial edema after 2-3 days, and subsequent development of facial palsy (grade 5 or 6). She had a mild lateral tongue keratosis, indurated on palpation. Taste sensations showed dysgeusia for sweet and salty. Results: Inflammatory, infectology and immunology tests were normal. Due to insufficient teeth, orthopantomogram was performed and showed oligodontia. Brain MRI showed normal brain and facial nerve path, but revealed right arachnoidal cyst with suspected glossopharyngeus compression, clinicaly asymptomatic. Tongue biopsy showed keratosis, acanthosis, edema and mononuclear infiltrates. She was treated with methylprednisolone and doxycycline, with physical therapy, and recovered completely. Conclusions: There is no standard therapy for this syndrome, etiology is still largely unknown. To our knowledge, this is the first report of MRS associated with oligodontia. The diagnosis of MRS is clinical and should be suspected in recurrent facial palsy.

Melkersson-Rosenthal sindrom; oligodoncija

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