Romberg Parry Syndrome Associated With Generalized Morphea: A Case Report
Journal Name:
- Abant Tıp Dergisi
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Abstract (2. Language):
Romberg Parry syndrome is an idiopathic neurocutaneous disorder which is rarely seen. Progressive facial hemiatro-phy may be seen in subcutaneous tissue, muscle and crani-ofacial bones. There are trauma, heredity, hypo-hyperactivation of Sympathetic nervous system, infections, autoimmunity, anomalies of trigeminal nerve and neuro-vascular disorders. Reconstructive surgery technical, lipo injection, silicon implantation, methotrexate, steroid, UVA-1 and PUVA can be used in treatment. Our case was 6 years old girl presented with multiple white plaques on her body and extremities and hemifacial atrophies on her zygomatic and temporomandibular area. Laboratory and radiologic investigations were unremarkable. Diagnosis was Romberg Parry syndrome. Cases of Romberg Parry syndrome are generally associated with linear scleroderma in literature. We decided to report this case because Romberg Parry syndrome is very rare and our case was associated with generalized morphea.
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Abstract (Original Language):
Romberg Parry sendromu nadir görülen ve idiopatik nörokutanöz bir bozukluktur. Subkutan doku, kas ve kra-niofasyal kemiklerde progresif fasyal hemiatrofi görülebil-ir. Etiyolojisinde travma, heredite, sempatik sinir sistemi hipo-hiperaktivitesi, enfeksiyonlar, otoimmunite, trigemi-nal sinir anomalileri ve nörovasküler bozuklukları vardır. Tedavide rekonstruktif cerrahi teknikleri, lipo enjeksiyon, silikon implantasyonu, metotrexate, steroid, UVA-1 ve PUVA kullanılabilir. Olgumuz 2 yıldır gövdede ve ekstrem-itelerde çok sayıda beyaz renkte plakları, zigomatik ve temporomandibular alanda hemifasyal atrofisi olan 6 yaşında kız çocuğuydu. Laboratuvar ve radyolojik tetki-klerinde özellik yoktu. Romberg Parry sendromu tanısı konuldu. Literatürdeki Romberg Parry sendromu olguları genellikle lineer skleroderma ile ilişkilidir. Romberg Parry sendromu nadir görüldüğü ve olgumuz generalize morfea ile ilişkili olduğu için bu olguyu sunmaya karar verdik.
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