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Parry-Romberg syndrome
Elizabeth Lazaridou1, Christina Giannopoulou, Zoi Apalla
1First Department of Dermatology and Venereology, Aristotle University Medical School, Thessaloniki, Greece.
Background:
Parry-Romberg syndrome (PRS) or idiopathic hemifacial atrophy is a rare neurocutaneous syndrome. It is characterized by slowly progressive atrophy, located on one side of the face, primarily involving the skin, fat and connective tissue. PRS seems to overlap with "en coupe de sabre" morphea.
Main Observations:
We present a case of hemifacial atrophy in a 14-year-old boy treated with topical calcipotriol-betamethasone ointment. The diagnosis of PRS was established mainly based on the clinical findings and histological picture. The time to diagnosis was almost 9 years, similar to the mean time reported in the literature.
Conclusions:
Understanding the pathogenesis and stopping disease progression is important as it can cause severe disfigurement and has neurological and psychiatric complications. Not much is known about the efficacy of agents used in the treatment of this syndrome making treatment decision very difficult. Possible complications, pathophysiology and therapeutic options are being discussed.
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