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.
Linear lichen planus is a rare variety of lichen planus characterized by lesions following the lines of Blaschko.Anterior cervical hypertrichosis is a rare and unrecognized form of local congenital hypertrichosis. It is characterized by the presence of a tuft of terminal hair on the anterior cervical region. We report an exceptional association of these two entities.
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