Paediatria Croatica, Vol. 57 No. 2, 2013.
Meeting abstract
A CASE OF MELKERSSON-ROSENTHAL SYNDROME ASSOCIATED WITH OLIGODONTIA IN A FIFTEEN-YEAR-OLD GIRL
Goran Krakar
; Department of Pediatric Neurology, Children’s Hospital Zagreb, Klaićeva 16, Zagreb, Croatia
Borut Marn
; Pediatric ENT Department, Children’s Hospital Zagreb, Klaićeva 16, Zagreb, Croatia
Nives Pustišek
; Department of Pediatric Dermatology, Children’s Hospital Zagreb, Klaićeva 16, Zagreb, Croatia
Monika Ulamec
; Clinical Department of Pathology, Sestre milosrdnice University Hospital Center, Vinogradska 29, Zagreb, Croatia
Tomislav Gojmerac
; Department of Pediatric Neurology, Children’s Hospital Zagreb, Klaićeva 16, Zagreb, Croatia
Vlatka Mejaški Bošnjak
; Department of Pediatric Neurology, Children’s Hospital Zagreb, Klaićeva 16, Zagreb, Croatia
Abstract
We report a 15-year old girl with Melkersson-Rosenthal syndrome associated with oligodontia. Melkersson-Rosenthal syndrome is a rare neuromucocutaneous disorder of uncertain etiology causing localized edema, inflammation of 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. Our patient was a 15-year-old girl with recurrent facial palsy (at the age 6, 8 and two times at the age of 15), three times left-sided and once right-sided. The 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 mild lateral tongue keratosis, indurated on palpation. Taste sensation showed dysgeusia for sweet and salty. Inflammatory, infectology and immunology tests were normal. Due to deficient or missing teeth, orthopantomogram was performed and showed oligodontia. Brain magnetic resonance imaging showed normal brain and facial nerve path, but revealed right arachnoidal cyst with suspected glossopharyngeus compression, clinically asymptomatic. Tongue biopsy showed keratosis, acanthosis, edema and mononuclear infiltrates. She was treated with methylprednisolone and doxycycline, along with physical therapy, and recovered completely. There is no standard therapy for this syndrome, its etiology still being largely unknown. To our knowledge, this is the first report of Melkersson-Rosenthal syndrome associated with oligodontia. The diagnosis of Melkersson-Rosenthal syndrome is clinical and should be suspected in recurrent facial palsy.
Keywords
Hrčak ID:
105092
URI
Publication date:
25.6.2013.
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