Ömer Faruk ELMAS, Şevki ÖZDEMİR, Şule BİLİCİ, Haticetül Kübra EFE, Okan KIZILYEL, Mahmut Sami METİN

Generalize Morfea İle İlişkili Romberg Parry Sendromu: Bir Olgu Sunumu

Romberg Parry sendromu nadir görülen ve idiopatik nörokutanöz bir bozukluktur. Subkutan doku, kas ve kraniofasyal kemiklerde progresif fasyal hemiatrofi görülebilir. Etiyolojisinde travma, heredite, sempatik sinir sistemi hipo-hiperaktivitesi, enfeksiyonlar, otoimmunite, trigeminal 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 ekstremitelerde çok sayıda beyaz renkte plakları, zigomatik ve temporomandibular alanda hemifasyal atrofisi olan 6 yaşında kız çocuğuydu. Laboratuvar ve radyolojik tetkiklerinde 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.

Anahtar Kelimeler:

Romberg Parry sendromu, generalize morfea, atrofi

Romberg Parry Syndrome Associated With Generalized Morphea: A Case Report

Romberg Parry syndrome is an idiopathic neurocutanous disorder which is rarely seen. Progressive facial hemiatrophy may be seen in subcutanous tissue, muscle and craniofacial bones. There are trauma, heredity, hypo-hyperactivation of Sympathetic nervous system, infections, autoimmunity, anomalies of trigeminal nerve and neurovascular 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.

Keywords:

Romberg Parry syndrome, generalized morphea, atrophy,

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