Srinivasan Ranjini, Joy Shasta Theresa
Department of Pediatrics, St. John's National Academy of health sciences, Sarjapur Main Rd, John Nagar, Koramangala, Bengaluru 560034, Karnataka, India.
Oxf Med Case Reports. 2020 Jan 31;2020(1):omz127. doi: 10.1093/omcr/omz127. eCollection 2020 Jan.
Parry-Romberg syndrome is a rare degenerative disorder causing progressive atrophy of skin and soft tissues of the face and neck, which is usually unilateral. The mean age of onset is usually in the second decade of life and the disease causes functional, aesthetic and psychological disabilities in the affected individual. We present a 14-year-old boy with this disorder. The diagnosis was based on clinical characteristics. A multidisciplinary team approach involving rheumatologists, dermatologists, maxillofacial surgeons, dentists and psychologists is required for the management of this problem, which is mainly targeted at controlling active inflammation with the use of immunosuppressive agents in addition to possible surgical correction of repositioning of adipose tissue that is lost due to atrophy.
帕里-罗姆伯格综合征是一种罕见的退行性疾病，可导致面部和颈部皮肤及软组织进行性萎缩，通常为单侧性。发病的平均年龄通常在人生的第二个十年，该疾病会给受影响个体带来功能、美学和心理方面的残疾。我们报告一名患有此疾病的14岁男孩。诊断基于临床特征。对于这个问题的管理，需要一个由风湿病学家、皮肤科医生、颌面外科医生、牙医和心理学家组成的多学科团队方法，其主要目标是除了对因萎缩而丢失的脂肪组织进行可能的手术复位矫正外，还使用免疫抑制剂来控制活动性炎症。
