Acquired Localized Cutis Laxa due to Increased Elastin Turnover.
Rie Harboe Nygaard1, Scott Maynard2, Peter Schjerling1
1Institute of Sports Medicine, Department of Orthopedic Surgery, Bispebjerg Hospital, Copenhagen, Denmark; Center for Healthy Aging, Copenhagen, Denmark.
Case Reports in Dermatology
|June 14, 2016
Summary
This study investigates acquired localized cutis laxa, a rare skin condition. Despite a lack of elastic fibers, affected skin showed increased elastin production, suggesting a defect in deposition rather than synthesis.
Area of Science:
- Dermatology and Molecular Biology
Background:
- Cutis laxa is a rare genetic disorder causing skin laxity due to elastin defects.
- Acquired forms of cutis laxa are less understood, particularly localized presentations.
Observation:
- A case of adult-onset, upper-body localized cutis laxa was studied.
- Skin biopsies revealed a lack of elastic fibers in affected areas.
- Affected skin showed higher elastin mRNA and RNA levels.
Findings:
- Electron microscopy confirmed absent elastin fibers in affected skin.
- Increased elastin mRNA and protein levels were observed in affected skin.
- Fibroblasts from affected skin produced elastin and exhibited enhanced stress resistance.
Implications:
- This case suggests acquired cutis laxa may involve impaired elastin deposition, not reduced synthesis.
- Understanding elastin metabolism defects is crucial for treating skin laxity disorders.
- Further research into extracellular matrix deposition mechanisms is warranted.
Related Concept Videos
01:12Elastin is Responsible for Tissue Elasticity
3.3K
Elastic fiber contains the protein elastin along with lesser amounts of other proteins and glycoproteins. The main property of elastin is that it will return to its original shape after being stretched or compressed. Elastic fibers are prominent in elastic tissues found in skin and the elastic ligaments of the vertebral column.
Ligaments and tendons are made of dense regular connective tissue, but in ligaments not all fibers are parallel. Dense regular elastic tissue contains elastin fibers and...
Ligaments and tendons are made of dense regular connective tissue, but in ligaments not all fibers are parallel. Dense regular elastic tissue contains elastin fibers and...
3.3K
01:23Skin Diseases and Disorders
6.1K
Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
6.1K
01:15Reticular Dermis
5.4K
The papillary and reticular dermis are the two layers of the dermis. They are made of connective tissue with fibers of collagen extending from one to the other, making the border between the two somewhat indistinct. The dermal papillae extending into the epidermis belong to the papillary layer, whereas the dense collagen fiber bundles below belong to the reticular layer.
Reticular Layer
Underlying the papillary layer is the much thicker reticular layer, composed of dense, irregular connective...
Reticular Layer
Underlying the papillary layer is the much thicker reticular layer, composed of dense, irregular connective...
5.4K
01:11Papillary Dermis
6.6K
Dermis
The dermis might be considered the "core" of the integumentary system, as distinct from the epidermis and hypodermis. It contains blood and lymph vessels, nerves, and other structures, such as hair follicles and sweat glands. The dermis is made of two layers of connective tissue that comprise an interconnected mesh of elastin and collagenous fibers, produced by fibroblasts.
Papillary Layer
The papillary layer is made of loose, areolar connective tissue, which means the collagen...
The dermis might be considered the "core" of the integumentary system, as distinct from the epidermis and hypodermis. It contains blood and lymph vessels, nerves, and other structures, such as hair follicles and sweat glands. The dermis is made of two layers of connective tissue that comprise an interconnected mesh of elastin and collagenous fibers, produced by fibroblasts.
Papillary Layer
The papillary layer is made of loose, areolar connective tissue, which means the collagen...
6.6K
01:12Renewal of Skin Epidermal Stem Cells
3.2K
The skin is divided into epidermis, dermis, and hypodermis, the skin's outermost, middle, and inner layers. The human epidermal layer regularly undergoes renewal, where old, dead cells are replaced by new cells. Epidermal stem cells or EpiSCs divide and differentiate to restore the lost cells. For the renewal process, some EpiSCs continuously self-renew. In contrast, few others differentiate into transit-amplifying cells, which later form prickle or spinous cells, followed by granular...
3.2K
01:19Clinical Applications of Epidermal Stem Cells
3.4K
Epidermal stem cells (EpiSCs) are mainly located at the basal layer of the epidermis. These cells repair minor injuries of the skin and replace dead skin cells. However, EpiSCs’ cannot heal severe wounds such as major burns or those from diabetes or hereditary disorders. In such cases, culturing the epidermal stem cells from the patient is possible and has yielded successful treatment options, such as laboratory-grown skin grafts. These grafts are synthesized using a patient’s own...
3.4K