A Tale of Two Diseases: Parry Romberg Syndrome and En Coup De Sabre: A Case Report
Keywords:
Parry Romberg syndrome, hemifacial atrophy, en coup de sabre morpheaAbstract
Background: Parry Romberg syndrome is a very rare disorder of unknown etiology. A degenerative acquired
disorder characterized by hemifacial atrophy. It can present with skeletal, dental and soft tissue abnormalities
with or without CNS symptoms. Majority of the affected patients have manifestations of the disease in the first
two decades of life. Many cases have been documented having combination of Parry Romberg syndrome and
another form of localized scleroderma- en coup de sabre morphea. Histological features of the disease include fibrosis
with atrophy of adipose tissue and lack of any inflammatory infiltrate. Limited treatment options make the
disease difficult to manage.
Case presentation: We present a case of an otherwise perfectly healthy, 12 years old girl with early features of
hemi-facial atrophy and en coup de sabre morphea.
Conclusion: Both Parry Romberg syndrome and en coup de sabre morphea have many localized and systemic
associations. In many case reports neurological and dental abnormalities have been described. In our case patient
did not appear to have any of these.
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Copyright (c) 2018 Saqib Hasnain, Muhammad Erfan, Syed Afaq Ahmed, Ikram ullah khan, Saqib Hasnain
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