Parry-Romberg, a neurocutaneous syndrome: presentation of three cases
Main Article Content
Keywords
Facial Hemiatrophy, Combined Modality Therapy, Parry Romberg Syndrome
Abstract
Background: Parry-Romberg syndrome is characterized by progressive hemiatrophy of the skin, subcutaneous tissue, muscle and bones of the skull. Its incidence is low, with a progressive and slow course. Its etiology is unknown, but it has been associated with several factors. Its clinical presentation involves dermatological, musculoskeletal and neuropsychiatric manifestations. The treatment consists of medical and surgical strategies. The use of steroids, alone or in combination with immunomodulators, has the objective of slowing down progression. The surgical treatment lies in facial reconstruction or volumetric regeneration, to correct the appearance and function of facial structures. The objective is to show three cases of Parry-Romberg syndrome with the representative characteristics of the disease.
Clinical cases: 1) A 41-year-old woman with atrophy and right supraciliary hypochromia associated with seizures and headache. 2) A 43-year-old woman with parietal deformation and right supraciliary atrophy, associated with facial paralysis, depression and headache. 3) A 36-year-old woman with right hemifacial atrophy associated with ocular involvement and headache. The indicated treatment was based on a systemic steroid plus a cytostatic agent. The surgical treatment was evaluated according to the affection of facial structures.
Conclusions: Parry-Romberg syndrome is a rare disease, characterized by progressive atrophy. Clinical manifestations determine the diagnosis and early start of medical and surgical treatment.
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