At the same time, recirculating flow into the aneurysm sac is amongst the most important factors that lead to further expansion of aneurysm and its rupture [27, 28], inducing a profound shift of the artery wall towards elastolysis
, inflammation, and oxidative stress.
ECM degradation enzymes, such as MMPs and cathepsins, are produced by macrophages and result in elastolysis
and alveolar tissue damage [179-182].
The differential diagnoses mainly include anetoderma, mid-dermal elastolysis
, focal dermal hypoplasia, lupus panniculitis, and localized scleroderma.
(MDE) is a rare disease of dermal connective tissue, described for the first time by Shelley and Wood in 1977, as "wrinkles due to idiopathic loss of elastic tissue of the middermis" .
These changes include mast cell degranulation and the presence of activated macrophages in association with mid-dermal elastolysis
. (23) When the lesions become become clinically visible, collagen bundles start showing structural alterations, fibroblasts become prominent, and mast cells are absent.
Type 3 includes patients of MKS with evidence of extrapulmonary elastolysis
. Persons with Type 4 MKS have no clear predisposing factors.
DISCUSSION: (1, 2, 3) Cutis laxa (Latin for loose or lax skin) also known as Generalized Elastolysis
/ Dermatomegaly / Dermatolysis / Dermatochalasia / Generalized elastorrhexis / Pachydermatocele is an Orphan disease (a very rare disease) related to a heterogeneous connective tissue disorder in which the skin becomes inelastic and hangs loosely in folds.
(13) A characteristic finding is the presence of extensive collagenolysis and elastolysis
, resulting in "floating fat" separated from the surrounding reticular dermis and the pannicular septa.
For example, aging causes the loss of elastin natural properties, and thus it becomes susceptible to deposition of calcium and elastolysis
. Enhanced decomposition of insoluble elastin in the vascular wall leads to the appearance of peptides in the blood stream, whereas anti-elastin antibodies are created in response to them .
Then activation of matrix metalloproteinase 2 causes elastolysis
of elastin and fibrillar collagen in arterial media.
On the other hand, genetic factors such as Marfan's syndrome, vascular Ehlers-Danlos syndrome, bicuspid aortic valve, and familial aortic dissection contribute to a compromised intima and aortic dissection through differentiation of the vascular smooth muscle cells and enhanced elastolysis
of the aortic wall components .
Collagen bundles appear to unravel, and the individual elastic fibers show signs of elastolysis
. The net effect of these fibrous rearrangements and alterations is a dermis that is less stretchable, less resilient, more lax, and prone to wrinkling.