Cutis laxa

Contents

What is it skin lax?

Cutis laxa (Latin for loose or lax skin) is rare, inherited, or acquired connective tissue disorder characterized by loose skin that lacks elasticity. The way the condition occurs is different depending on how it is inherited or acquired. Cutis laxa is also known as generalized elastolysis.

Connective tissue is the supporting framework of the tissue body consisting of strands of collagen, elastic fibers between muscles and around muscle groups and blood vessels, and single cells.

Who gets lax skin and why?

Most cases of cutis laxa are inherited. There are several ways to genetic heritage: sex-linked, autosomal dominant and autosomal recessive. The recessively inherited form is the most common and also the most severe. Recently discovered a faulty gene in chromosome 5 has been associated with problems in normal elastogenesis and is believed to be the cause of autosomal recessive cutis laxa.

Sex linked	The defective gene is transported in one of the sexes. chromosomes, in this case it is on the X chromosome
Autosomal dominant	It only takes one copy of the faulty gene for the disease to be present. A child has a 50% chance of inheriting the gene from either parent.
Autosomal recessive	Two copies of the faulty gene must be inherited for the disease to be present. A child must inherit a faulty gene from both parents. The risk of transmitting an autosomal recessive disease is 25% for each pregnancy.

Acquired complexion lax can develop at any age, but it often begins in adulthood. It can occur spontaneously or in 50% cases develop after episodes of urticaria or angioedemaextensive inflammatory skin disease (eg. eczema, erythema multiform, blisters eruption) or hypersensitivity reactions to penicillin or other drugs.

The actual underlying cause of cutis laxa is unknown. Several mechanisms appear to be involved in the reduction of elastic fibers.

What are the clinical characteristics of cutis laxa?

The clinical features of cutis laxa are different depending on how the condition is inherited.

Heritage	Clinical features
X-linked	Loose skin Loose joints (joint hyperextensibility) Bone abnormalities such as aquiline nose, pigeon breast and funnel Frequent loose stools and urinary tract obstructions Mild mental retardation
Autosomal dominant	Skin changes can start at any age. Hanging loose skin may be the only feature Facial participation is universal. Systemic participation is rare
Autosomal recessive (types I and II)	Symptoms and signs generally begin in childhood Loose skin Bone abnormalities (eg, delayed union of skull bones, hip dislocation, spinal curvature) Emphysema (lung disease causing dyspnea) Hernias Diverticula (bags) in the esophagus, duodenum, and bladder. Aortic aneurysm (bulging aorta, the main artery leaving the heart) Type i: Severe form Present at birth Emphysema early in life Type II Growth retardation Generally present at birth Severe on hands, feet and abdomen Bilateral congenital hip dislocation

Heritage

Clinical features

X-linked

Loose skin
Loose joints (joint hyperextensibility)
Bone abnormalities such as aquiline nose, pigeon breast and funnel
Frequent loose stools and urinary tract obstructions
Mild mental retardation

Autosomal dominant

Skin changes can start at any age.
Hanging loose skin may be the only feature
Facial participation is universal.
Systemic participation is rare

Autosomal recessive (types I and II)

Symptoms and signs generally begin in childhood
Loose skin
Bone abnormalities (eg, delayed union of skull bones, hip dislocation, spinal curvature)
Emphysema (lung disease causing dyspnea)
Hernias
Diverticula (bags) in the esophagus, duodenum, and bladder.
Aortic aneurysm (bulging aorta, the main artery leaving the heart)

Type i:

Severe form
Present at birth
Emphysema early in life

Type II

Growth retardation
Generally present at birth
Severe on hands, feet and abdomen
Bilateral congenital hip dislocation

The characteristics of acquired loose skin are mainly loose skin, especially in areas of the anterior inflammation. It has also happened in association with complement deficiency (C3 and C4), systemic lupus erythematosus, sarcoidosis, multiple myeloma, and systemic amyloidosis.

Characteristics of lax complexion skin

Any part of the body can be affected. In inherited forms of loose skin, the appearance of loose skin is most prominent around the eyes, face, neck, shoulders, and thighs. The characteristics of the skin include:

Loose, inelastic and hanging skin folds
Loose skin that hangs around the face and neck, giving you the look of a hound and a patient who looks much older than he really is
Reduced elastic recoil when the skin is stretched.

How is cutis laxa diagnosed?

Cutis laxa is generally diagnosed clinically, due to its unique clinical characteristics. the pathology of loose complexion on the skin biopsy It can be useful

What is the treatment for cutis laxa?

There is no specific treatment for cutis laxa or to prevent disease progression. Treatment is aimed at managing any complications that may arise from the involvement of the associated internal organs.

Cosmetic surgery can be performed to reduce redundant skin folds, but often only a temporary benefit. Patients with lax skin, particularly those with the autosomal dominant form, generally have a normal life expectancy.