Parry Romberg syndrome with en coup de sabre: A report of a rare case

Abstract

The basis of the problem: Parry Romberg syndrome (PRS) is a rare developmental disorder manifesting as a slow and progressive atrophy of the face which is often unilateral, hence also termed as rpogressivehemifacial atrophy. The extent of the atrophy may vary, involving the superficial skin extending upto the underlying bone. The clinical features of PRS include craniofacial, neurologic, ophthalmic and dermatological manifestations, which result in various functional and psychological problems. PRS and linear scleroderma belong to the same disease spectrum. There can be demarcating line between the normal and abnormal skin seen in PRS, termed as "en coup de sabre". PRS is most commonly seen in females and involves the left side of the face. Method: We hereby report a case of PRS in a young girl presenting with en coup de sabre appearance on the right side of face. Results: Microsurgical facial reconstruction of the affected side is known to be the gold standard method for correction of the facial symmetry. In our case, the patient is currently kept under long term follow up and cosmetic surgical treatment will be planned once the atrophy attains stability. Conclusion: The association of PRS with linear scleroderma may present difficulty in its diagnosis. Hence, careful recording of the history and clinical examination with appropriate investigations can aid in establishing the correct diagnosis.

Keywords

• cone-beam computed tomography
• hemifacial atrophy
• maxillofacial abnormalities
• rare diseases