Similar hyperplasia of the vascular system can occur causing vascular malformations.
OVERGROWTH SYNDROME SKIN
The thick, firm, furrowed skin in this case is due to epidermal connective tissue hyperplasia.
These latter produce cerebriform (grooved deeply, like the cerebrum or outer layer of the brain) patterns in the skin. The skin also often shows areas of dermal hypoplasia, or areas with abnormal capillaries called telangiectatic nevi, or connective tissue nevi in the palms and the soles. This is the only lesion in this condition that remains stable with time. Keratinocytic nevi are frequent in this condition, occurring as velvety overgrowths of the skin, being found in at least half the people affected with Proteus syndrome. Its prevalence is very low, with less than one in a million being affected anywhere in the world. It is present at birth but manifests only after a few months, typically at 6 to 18 months, and worsens with age. It is a rare genetic condition in which some parts of the body start to grow too much, in a random manner.Īs a result of the uncontrolled proliferation of various tissues, as in the skin and the bones, with no apparent pattern, some body parts finally come to a size out of all proportion to the rest of the body.
Proteus syndrome has also been termed ‘elattoproteus syndrome’ or the ‘elephant man disease’. Liji Thomas, MD Reviewed by Susha Cheriyedath, M.Sc.