title: Congenital Volkmann syndrome and aplasia cutis of the forearm: a challenging differential diagnosis.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors: Division of Dermatology, Department of Specialized, Clinical and Experimental Medicine, University of Bologna, Bologna, Italy.
authors: Iria
authors: I
authors: Neri
authors: Division of Dermatology, Department of Specialized, Clinical and Experimental Medicine, University of Bologna, Bologna, Italy.
authors: Michela
authors: M
authors: Magnano
authors: Child Neurology and Psychiatry Unit, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.
authors: Antonella
authors: A
authors: Pini
authors: Division of Dermatology, Department of Specialized, Clinical and Experimental Medicine, University of Bologna, Bologna, Italy.
authors: Lorenza
authors: L
authors: Ricci
authors: Division of Dermatology, Department of Specialized, Clinical and Experimental Medicine, University of Bologna, Bologna, Italy.
authors: Annalisa
authors: A
authors: Patrizi
authors: Division of Dermatology, Department of Specialized, Clinical and Experimental Medicine, University of Bologna, Bologna, Italy.
authors: Riccardo
authors: R
authors: Balestri
mesh_terms: D003937:Diagnosis, Differential; D004476:Ectodermal Dysplasia / Q000175:diagnosis*; D005260:Female; D005542:Forearm; D006801:Humans; D007231:Infant, Newborn; D054061:Ischemic Contracture / Q000151:congenital* / Q000175:diagnosis*