Author: Dr Marius Rademaker, Dermatologist, Hamilton, New Zealand, 2008. Update by DermNet NZ Editor in Chief Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. December 2017. Copy edited by: Gus Mitchell.
What is a glomuvenous malformation?
Glomuvenous malformation is an unusual form of venous malformation, typically diagnosed at birth or later in infancy or childhood. Glomuvenous malformation is also known as glomangioma or glomangiomatosis.
Who gets glomuvenous malformation?
Although glomuvenous malformation may be sporadic, it may also be familial with an autosomal dominant pattern of inheritance (this is where half the children of an affected parent inherit the condition). An abnormal chromosome 1p21-p22 has been identified in these families, which results in inactivation of a protein called glomulin.
What are the clinical features of glomuvenous malformation?
A glomuvenous malformation presents as a localised or segmental collection of soft, pink, red, or blue nodules or coalescingplaques. They may arise anywhere on the skin and may rarely affect mucous membranes. These vascular lesions are less compressible than other forms of venous malformation.
The lesions tend to get more widespread, thicker, and deeper in colour with age. Although the nodules start off painless, they may later become tender to touch.
How is glomuvenous malformation diagnosed?
Glomuvenous malformation may be suspected by its clinical appearance; however, biopsy may also be undertaken. Upon histology, glomuvenous malformation resembles venous malformation with large, dilated, thin-walled veins in the dermis and subcutaneous tissue. Clusters of alpha-actin-positive glomus cells (benigntumour cells in the soft tissue) are found lining the venous spaces.
Wassef M, Blei F, Adams D, et al; ISSVA Board and Scientific Committee. Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 2015 Jul; 136: e203-14. DOI:10.1542/peds.2014-3673. Epub 2015 Jun 8. Review. PubMed PMID: 26055853. Journal.