Cutis laxa

Web Cutis Laxa CL is a rare disorder of connective tissue that affects only about 400 families worldwide or 1 in every 2000000 babies. Cutis laxa may be caused by mutations in the genes.

Cutis Laxa Pathology Dermnet

Patients develop a prematurely aged.

. Web Cutis laxa is a disorder of connective tissue which is the tissue that provides structure and strength to the muscles joints organs and skin. Cutis laxa is also known as generalised elastolysis. Web Cutis laxa is a rare disorder that affects males and females in equal numbers.

Autosomal dominant CL autosomal recessive CL and. Web Cutis laxa is the general name for a group of rare disorders that affects your connective tissue. ELN ATP6V0A2 ATP7A FBLN4 FBLN5 and PYCR1.

This tissue gives your muscles joints skin and organs structure. Most types of cutis laxa. Web Cutis laxa is a connective tissue disorder characterized by skin that is sagging and not stretchy.

This can be related to decreased elastin synthesis or structural defects in the extracellular matrix. The skin often hangs in loose folds causing the face and other parts of the body to have a droopy appearance. Cutis laxa can also affect connective tissue in other parts of the body including the heart blood vessels joints intestines and lungs.

Web Cutis laxa CL is a group of rare diseases of connective tissue characterized by redundant skin with loss of elasticity and premature aging. 1 Heritable forms have variable transmissions and clinical expressions. Three major groups are individualized based on the mode of inheritance.

Web Cutis laxa is associated with deficient or absent elastin fibers in the extracellular matrix. Web Cutis laxa Latin for loose or lax skin is a rare inherited or acquired connective tissue disorder characterised by loosely hanging skin that lacks any elasticity. Generally cutis laxa is characterized by saggy loose wrinkly and inelastic skin especially around the face neck arms legs and torso.

Connective tissue also referred to as the extracellular matrix provides the structural framework for many parts of the body including skin muscles joints blood vessels and even internal organs. Web Cutis laxa or elastolysis is a rare inherited or acquired connective-tissue disorder in which the skin becomes inelastic and hangs loosely in folds. Web Cutis Laxa is a rare disorder that causes a defect or an insufficiency of the bodys connective tissue affecting the normal structural framework of the skin muscles joints and sometimes internal organs.

The way in which the condition presents itself is different according to how it is inherited or acquired. The disorder has been reported in approximately 400 families worldwide. Most cases are inherited but some are acquired which means they do not appear to be caused by genetic variations.

Cutis laxa is estimated to affect 1 in 1000000 individuals in the general population.

Cutis Laxa Springerlink

Cutis Laxa Wikidoc

Indian Pediatrics Editorial

Genotype Phenotype Spectrum Of Pycr1 Related Autosomal Recessive Cutis Laxa Sciencedirect

Congenital Cutis Laxa

John Libbey Eurotext European Journal Of Dermatology Acquired Localized Cutis Laxa Occurring On The Face After Delivery

Cutis Laxa An Overview Sciencedirect Topics

Patient 2 Cutis Laxa Notably In The Neck Lipodystrophy Prominent Download Scientific Diagram

Progeroide Autosomal Rezessive Cutis Laxa Syndrome Springerlink

Figure 1 From Generalized Acquired Cutis Laxa Associated With Multiple Myeloma With Biphenotypic Igg L And Iga K Gammopathy Following Treatment Of A Nodal Plasmacytoma Semantic Scholar