Runne U, Fasshauer K
Hautarzt. 1977 Jan;28(1):10-7.
Six patients with facial hemiatrophy (H.f) were thoroughly examined by clinical and laboratory investigations. Two were found to have idiopathic and four facial hemiatrophy due to different types of localized scleroderma. In all cases a generalized myopathy was present, demonstrated by electromyographical, histological, and biochemical means. In none of these cases a hereditary or neurological cause for the facial hemiatrophy was found. However, in two cases autoantibodies against nuclei and muscle were repeatedly obtained. No prolongation of sensory or pain chronaxy occurred in either the patients with sclerodermal or idiopathic facial hemiatrophy. These observations suggest that facial hemiatrophy can originate (a) in a localized scleroderma in the involved part of the face, (b) in an ipsilateral "sclérodermie en coup de sabre", or (c) in the coexistence of both. There is a close pathological and physiological correlation between idiopathic and sclerodermal facial hemiatrophy. In both forms of facial hemiatrophy the disease involves skeletal muscle tissue systematically. This myopathy is similar to that of progressive scleroderma, far exceeding the limited muscular involvement of localized scleroderma. Sclerodermal facial hemiatrophy can be associated with autoimmune phenomena.
对6例面部半侧萎缩（H.f）患者进行了全面的临床和实验室检查。发现2例为特发性，4例面部半侧萎缩是由不同类型的局限性硬皮病引起的。所有病例均存在全身性肌病，通过肌电图、组织学和生化方法得以证实。在这些病例中，均未发现面部半侧萎缩的遗传或神经学原因。然而，有2例患者反复检测到抗核和抗肌肉自身抗体。硬皮病性或特发性面部半侧萎缩患者均未出现感觉或疼痛时值延长。这些观察结果表明，面部半侧萎缩可能起源于（a）面部受累部位的局限性硬皮病，（b）同侧“剑伤性硬皮病”，或（c）两者并存。特发性和硬皮病性面部半侧萎缩之间存在密切的病理和生理相关性。在两种形式的面部半侧萎缩中，疾病均系统性地累及骨骼肌组织。这种肌病类似于进行性硬皮病，远远超过局限性硬皮病有限的肌肉受累程度。硬皮病性面部半侧萎缩可能与自身免疫现象有关。
