Division of Dermatology, Department of Specialized, Clinical and Experimental Medicine, University of Bologna, Bologna, Italy.
Child Neurology and Psychiatry Unit, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.
JAMA Dermatol. 2014 Sep;150(9):978-80. doi: 10.1001/jamadermatol.2014.111.
Differential diagnosis between congenital Volkmann ischemic contracture (CVIC) and unilateral aplasia cutis congenita (ACC) type VII of the forearm presents a clinical challenge. Both diseases share the same clinical presentation characterized by a stellate ulceration over the upper extremities and reported association with neuromuscular defects, but the diagnostic criteria to differentiate these 2 entities remain unclear.
A newborn girl presented with an ulceration of the left forearm associated with an apparent neurological impairment. On the basis of the suspected neurological involvement, a diagnosis of CVIC was initially considered, but because the neurological evaluation excluded paralysis, our final diagnosis was ACC type VII.
In our opinion, CVIC should be considered a particular form of ACC in which an external noxa affects the forearm, increasing the intracompartmental pressure and leading to muscle and nerve ischemia. Therefore, we propose that the definition of Volkmann ischemic contracture should be maintained only for the acquired forms with an evident etiology and that Frieden's classification scheme for ACC type VII needs to be reformulated.
先天性沃尔曼缺血性挛缩（CVIC）和前臂单侧先天性表皮发育不全（ACC）VII 型之间的鉴别诊断具有临床挑战性。这两种疾病具有相同的临床表现，特征为上肢出现星状溃疡，并伴有神经肌肉缺陷的报道，但区分这两种疾病的诊断标准仍不清楚。
一名新生女婴出现左前臂溃疡，并伴有明显的神经功能障碍。基于疑似神经受累，最初考虑 CVIC 的诊断，但由于神经评估排除了瘫痪，我们的最终诊断为 ACC 型 VII 型。
我们认为，CVIC 应被视为 ACC 的一种特殊形式，其中外部病因会影响前臂，增加腔室内压力，导致肌肉和神经缺血。因此，我们建议仅将具有明显病因的获得性形式的沃尔曼缺血性挛缩的定义保留下来，而弗里登的 ACC 型 VII 分类方案需要重新制定。
