The Asymmetrical Face of a Potential Systemic Disease: A Pattern to Recognize

Parry-Romberg syndrome (PRS) is a rare disorder characterized by a progressive atrophy of the skin, subcutaneous tissue, muscle, and bone of the hemiface, being more common in females. The etiology is unknown, and symptomatology is variable, beginning in the first two decades of life and evolving over 20 years or more. There is no specific treatment, and prognosis depends on the severity of lesions. We report the case of a 17-years-old male who presented with a painless, chronic cutaneous lesion on his left hemiface, resulting in malar depression, not responding to topical corticosteroid treatment. The neurological examination revealed left hemiface atrophy involving the nasolabial fold and malar area and tapered fingers. Autoimmune tests yielded normal results, and a brain magnetic resonance imaging (MRI) showed a reduction of left infraorbital fat tissue, with no endocranial lesions. He remains under surveillance, without a specific therapeutic intervention.