%0 Journal Article
%T Lower limb onset Parry¨CRomberg syndrome: an unusual presentation of a
rare disease
%A Rajendra Singh Jain
%A Sunil Kumar
%A Trilochan Srivastava
%J Archive of "Oxford Medical Case Reports".
%D 2016
%R 10.1093/omcr/omw031
%X Parry¨CRomberg syndrome (PRS) is characterized by progressive degeneration and atrophy of the cutaneous, subcutaneous connective tissues, muscles and bones. Classically, PRS is restricted to unilateral face but in 20% of patients may extend to other parts of the body including ipsilateral or contralateral arms, trunk and legs. We report a case of 24-year-old male who presented with insidious onset, gradually progressive deformity and muscle wasting of right lower limb followed by right side of face and chest for 8 years. The right side of the face showed hemiatrophy, coup de sabre and deviation of nose and chin toward the same side. The magnetic resonance imaging showed atrophy of right lower limb. Computed tomography with 3D facial reconstruction revealed atrophy of facial bones on right side. He was managed with physiotherapy and symptomatic treatment and planned for facial and ankle reconstructive surgery on follow-up
%U https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5782432/