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[Parry-Romberg progressive facial hemiatrophy and localized scleroderma. Nosologic and pathogenic problems]
C Auvinet1, A Glacet-Bernard, G Coscas
1Clinique Ophtalmologique Universitaire de Créteil, Université de Paris XII, France.
Journal Francais D'Ophtalmologie
|January 1, 1989
Summary
This case report details a 65-year-old woman with Parry-Romberg syndrome, experiencing facial pain and diplopia due to progressive hemifacial atrophy. The study discusses potential links between this condition, scleroderma, and autonomic nervous system disorders.
Area of Science:
- Neurology
- Dermatology
- Ophthalmology
Background:
- Parry-Romberg syndrome, or progressive hemifacial atrophy, is a rare condition characterized by gradual loss of tissue on one side of the face.
- Its etiology remains poorly understood, with ongoing debate regarding its origins and potential associations.
Observation:
- A 65-year-old woman presented with a ten-year history of progressive hemifacial atrophy.
- Symptoms included facial pain, diplopia, severe enophthalmos, eyelid atrophy, alopecia, and skin changes consistent with scleroderma en coup de sabre.
- Ophthalmic findings revealed miosis, Horner's syndrome, and ocular motility disturbance.
Findings:
- The patient exhibited significant unilateral facial tissue loss and associated ophthalmic complications.
- Biomicroscopic examination of the anterior and posterior eye segments was unremarkable, ruling out certain intraocular pathologies.
- The case highlights the complex interplay of neurological, dermatological, and ophthalmological manifestations in Parry-Romberg syndrome.
Implications:
- This case underscores the need for a multidisciplinary approach in managing patients with Parry-Romberg syndrome.
- Further research into the relationship between hemifacial atrophy, scleroderma, and autonomic dysfunction is warranted.
- Aesthetic surgical intervention was proposed as a treatment option for the patient's facial disfigurement.