About Pseudoxanthoma elasticum-like papillary dermal elastolysis

What is Pseudoxanthoma elasticum-like papillary dermal elastolysis?

Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare skin disorder characterized by the breakdown of elastic fibers in the skin. It is characterized by yellow-brown papules and plaques on the neck, chest, and upper back. The condition is caused by a mutation in the ABCC6 gene, which is responsible for the production of an enzyme that helps maintain the structure of elastic fibers. PXE-PDE is often misdiagnosed as pseudoxanthoma elasticum, a genetic disorder that affects the elastic fibers in the skin, eyes, and cardiovascular system.

What are the symptoms of Pseudoxanthoma elasticum-like papillary dermal elastolysis?

The symptoms of Pseudoxanthoma elasticum-like papillary dermal elastolysis include:

-Skin Lesions that are yellowish-brown in color
-Lesions that are typically found on the neck, chest, and upper back
-Lesions that may be itchy or painful
-Lesions that may become raised or bumpy
-Lesions that may become scaly or crusty
-Lesions that may become larger over time
-Lesions that may be accompanied by other skin changes, such as discoloration or thinning of the skin

What are the causes of Pseudoxanthoma elasticum-like papillary dermal elastolysis?

The exact cause of Pseudoxanthoma elasticum-like papillary dermal elastolysis is unknown. However, it is believed to be caused by a combination of genetic and environmental factors. Genetic factors may include mutations in the genes responsible for the production of elastin, a protein that gives skin its elasticity. Environmental factors may include exposure to ultraviolet radiation, certain medications, and certain skin conditions.

What are the treatments for Pseudoxanthoma elasticum-like papillary dermal elastolysis?

1. Topical retinoids: Topical retinoids, such as tretinoin, can help reduce the appearance of the lesions.

2. Laser therapy: Laser therapy can help reduce the appearance of the lesions and improve skin texture.

3. Intralesional corticosteroids: Intralesional corticosteroids can help reduce inflammation and improve the appearance of the lesions.

4. Photodynamic therapy: Photodynamic therapy can help reduce the appearance of the lesions and improve skin texture.

5. Systemic medications: Systemic medications, such as hydroxychloroquine, can help reduce inflammation and improve the appearance of the lesions.

What are the risk factors for Pseudoxanthoma elasticum-like papillary dermal elastolysis?

1. Genetic predisposition: Pseudoxanthoma elasticum-like papillary dermal elastolysis is an inherited disorder caused by mutations in the ABCC6 gene.

2. Age: The disorder is most commonly seen in adults between the ages of 30 and 50.

3. Gender: Women are more likely to be affected than men.

4. Sun exposure: Prolonged sun exposure may increase the risk of developing the disorder.

5. Certain medications: Certain medications, such as isotretinoin, may increase the risk of developing the disorder.

Is there a cure/medications for Pseudoxanthoma elasticum-like papillary dermal elastolysis?

At this time, there is no known cure for Pseudoxanthoma elasticum-like papillary dermal elastolysis. Treatment is focused on managing symptoms and preventing complications. This may include topical corticosteroids, oral antihistamines, and photoprotection. In some cases, laser therapy may be used to reduce the appearance of lesions.