Congenital Volkmann syndrome and aplasia cutis of the forearm: A challenging differential diagnosis

Iria Neri, Michela Magnano, Antonella Pini, Lorenza Ricci, Annalisa Patrizi, Riccardo Balestri

Research output: Contribution to journalArticlepeer-review


IMPORTANCE 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.

OBSERVATIONS 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 AND RELEVANCE 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.

Original languageEnglish
Pages (from-to)978-980
Number of pages3
JournalJAMA Dermatology
Issue number9
Publication statusPublished - 2014

ASJC Scopus subject areas

  • Dermatology
  • Medicine(all)


Dive into the research topics of 'Congenital Volkmann syndrome and aplasia cutis of the forearm: A challenging differential diagnosis'. Together they form a unique fingerprint.

Cite this