Parry-Romberg syndrome

Also known as outlined Rpmberg's disease, facial unilateral dystrophy. Cause pathogenesis is still unclear. Likely to genetic diseases. Autonomic nervous system that is the central or peripheral damage caused unilateral facial nutrition obstacles all unilateral facial atrophy, subcutaneous adipose tissue and connective tissue atrophy was more apparent. 15% accompanied by hemispheric atrophy may be ipsilateral and contralateral or bilateral nature. Clinical features of the face of unilateral or all of an organization for atrophy. 3/5,20 about women before the age of onset of accounting for three-quarters of onset implicit attack. See more left, face more from the shrinking part, the uncertain pace of progress, generally about 2-10 years to develop into full-unilateral atrophy. 5% of the cases involving both sides of the face, in addition to facial atrophy, often involving soft palate, tongue and oral mucosa, see the ipsilateral neck, Heart to the trunk and limbs atrophy (about 10%). Side face sag Maung was elderly, and was in stark contrast to the healthy side, the hair, eyebrows, eyelashes often lost, a white, the skin on her forehead, etc.. Have facial pain or migraine, a rare sensory dysfunction, in addition to the affected sweat glands and lacrimal gland adjustment disorder, Visibility Horner's syndrome, a small number of patients with epilepsy, about half have paroxysmal EEG activities. Associated with brain atrophy may have hemiplegia, migraine body sensation, heminopia, such as aphasia. Differential diagnosis (1) systemic sclerosis (progressive systemic scle rosis) as a rheumatic disease, a type limited to the skin, and another - and offal. Standing between the age of 20-50 for women of childbearing age, the sex ratio is about 1:2~3. Chang first Raynaud's phenomenon (90%) or symmetry fingers swelling can be rigid, skin lesions are usually found in the fingers and hands or face, and that was swelling edema, no indentation (swelling period) Succeeding skin thickening stiffen as leather, Matt (hardening period), the final skin atrophy (shrinkage period) skin lesions gradually to the arm, neck, chest and abdomen spread. Facial normal striae disappear, and the face rigid, trismus, the hardening position pigmentation, from among stain. (2) surface-shoulder-facioscapulohumeral muscular dystrophy (Facioscapulohumeral mucul ar dystrophy) occurred in the young face of the slow muscle atrophy. special "myopathy face." slightly drooping eyelid, patterns and the amount of nasolabial disappear, and expressions of movement weak or disappeared, by the orbicularis oris muscle hard mast is thickened lips and eyes (cat face). With eyes closed, and do not blowing dark gills not, brachial, shoulders and face muscle atrophy, favors arm weakness, upper extremities are held at the scapula has wings like a breakthrough. Serum-induced acid phosphokinase (CPK), pyruvate kinase (PK) activity increased. (3), lipodystrophy (lipodystrophy) mostly females, more than 5 to 10 years after onset, often symmetrical distribution, progress has been slow. For the conduct of the disappearance of the subcutaneous fat or weight loss, rapid onset in the face, cheek and temporal Ru indentation of skin relaxation, deprived of the normal flexibility of Orbital deep, followed affect neck, shoulder, arm and torso. In some cases disease is confined to the face, or hemi-face, hemineglect body. Parry may confuse -Rombery's syndrome, However, the former biopsy only subcutaneous adipose tissue disappeared.