Extra-Cephalic Involvement in the Syndrome of Parry Romberg

Abstract

Parry-Romberg Syndrome (PRS) is an uncommon disorder characterized by a slowly and acquired progressive atrophy involving skin, soft tissue, cartilage, and bony structures. Accompanying atrophies of the other parts of the body are rarely reported. We report an extremely unusual case with PRS associated with contralateral lower extremity atrophy. KeywordsParry romberg syndrome, Facial haematrophy, Extra-cephalic involvement, Lower limb, Scleroderma IMAGE ARTICLEfacial mass demonstrated a discreet atrophy of the soft straight jaw parts without osseous involvement. The spinal teleradiography was in favor of dorso-lumbar scoliosis by inequality of the 2 lower limbs, with a 14 mm shortening of the left lower limb without apparent bone lesions.Electroencephalogram, ultrasound of supra-aortic trunks, and cerebral MRI were normal. A systemic treatment with corticosteroids of 0.5 mg/kg/day and Methotrexate at 12.5 mg/week was initiated. The progression was satisfactory, with the end of the progression of atrophy, within a follow-up of 18 months. A facial lipostructure for morphological restoration is expected after stopping growth.

Parry–Romberg syndrome associated with en coup de sabre in a patient from South Sudan – a rare entity from East Africa: a case report

“…It is characterized by slowly progressive facial hemiatrophy affecting all tissue layers from skin to bone and frequently (21–31%) overlaps with a condition known as linear scleroderma “ en coup de sabre .” It has a prevalence rate of 1 in 70,000 with no difference in rates between different ethnic groups. However, only four cases were reported from Africa in the English language literature: Gueye A, et al [14], Singh M, et al [15], Ilhame N, et al [16], and Elsaid N, et al [17] from Senegal, Libya, Morocco, and Egypt, respectively. It affects mainly females and usually involves the left side of the face.…”