Introducción: El Síndrome de Parry-Romberg es un trastorno neurocutáneo, caracterizado por hemiatrofia facial progresiva, su primer signo clínico la asimetría facial, se presenta frecuentemente manera simultánea con esclerodermia lineal. Presentación caso: Femenina 54 años edad, antecedente y alteración oftalmológica izquierda desde los 14 años. Discusión: inicia entre las primeras décadas vida...

Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bon...

A giant intracranial aneurysm was diagnosed in a 10-year-old girl when she developed a right abducens nerve palsy. The aneurysm was treated successfully. Six years later, however, she presented with a progressive en coup de sabre deformity, leading to the diagnosis Parry Romberg Syndrome (PRS), a rare diagnosis characterized by hemifacial atrophy of skin, subcutaneous tissue, skeletal muscle an...

BACKGROUND Linear scleroderma "en coup de sabre" (LSCS) usually affects one side of the face and head in the frontoparietal area with band-like indurated skin lesions. The disease may be associated with facial hemiatrophy. Various ophthalmological and neurological abnormalities have been observed in patients with LSCS. We describe an unusual case of LSC. CASE PRESENTATION A 23 year old woman ...

Parry-Romberg 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 ons...

A 52-year-old man presented with left hemifacial atrophy (figure 1) beginning at age 25. There were no neurologic symptoms. Neurologic examination showed no deficits, and the limbs were symmetric. Brain MRI demonstrated left cerebral hemiatrophy (figure 2) and lack of the ipsilateral soft facial tissue. Progressive facial hemiatrophy (PFH), or ParryRomberg syndrome, is a sporadic disease of unk...

This 45 years old lady complained of asymmetry of her face since the age of 15 years. She also complained of decreased sweating on the left side and an intermittent left hemicranial headache. Examination revealed marked atrophy of the left side of the face involving the skin, subcutaneous tissue and muscles of the orbital, maxillary and mandibular regions. There was no induration or tightening ...