About Dermatopathia pigmentosa reticularis
What is Dermatopathia pigmentosa reticularis?
Dermatopathia pigmentosa reticularis (DPR) is a rare, inherited skin disorder characterized by the presence of dark, pigmented patches on the skin. These patches are usually found on the trunk, arms, and legs, and may be accompanied by small, raised bumps. DPR is caused by a mutation in the gene responsible for producing the protein keratin-14, which is important for the structure and function of the skin. Symptoms of DPR can vary from person to person, but may include skin discoloration, itching, and scaling. Treatment for DPR is typically focused on managing symptoms and preventing further skin damage.
What are the symptoms of Dermatopathia pigmentosa reticularis?
The main symptom of Dermatopathia pigmentosa reticularis (DPR) is a reticulated pattern of hyperpigmentation on the skin. This pattern is usually symmetrical and may be seen on the trunk, arms, and legs. Other symptoms may include:
• Hyperpigmentation of the skin
• Hypertrichosis (excessive hair growth)
• Hyperkeratosis (thickening of the skin)
• Nail changes (thickening, splitting, or discoloration)
• Pruritus (itching)
• Photosensitivity (sensitivity to sunlight)
• Skin fragility (easily bruised or torn skin)
• Skin ulcerations (open sores)
• Scarring
What are the causes of Dermatopathia pigmentosa reticularis?
Dermatopathia pigmentosa reticularis (DPR) is a rare genetic disorder that affects the skin. It is caused by mutations in the GJB2 gene, which is responsible for the production of connexin 26, a protein that helps cells communicate with each other. Mutations in this gene can lead to a decrease in the production of connexin 26, resulting in the development of DPR. Other causes of DPR include mutations in the GJB3 gene, which is responsible for the production of connexin 30, and mutations in the GJB6 gene, which is responsible for the production of connexin 31.
What are the treatments for Dermatopathia pigmentosa reticularis?
The treatments for Dermatopathia pigmentosa reticularis (DPR) are limited. Treatment options include topical corticosteroids, topical calcineurin inhibitors, phototherapy, and systemic retinoids. Topical corticosteroids are used to reduce inflammation and itching. Topical calcineurin inhibitors, such as tacrolimus and pimecrolimus, are used to reduce inflammation and itching. Phototherapy, such as narrowband ultraviolet B (UVB) or excimer laser, can be used to reduce the appearance of the lesions. Systemic retinoids, such as isotretinoin, can be used to reduce the size and number of lesions.
What are the risk factors for Dermatopathia pigmentosa reticularis?
The exact cause of Dermatopathia pigmentosa reticularis (DPR) is unknown, but it is believed to be an inherited disorder. Risk factors for developing DPR include having a family history of the condition, being of a certain ethnic background (such as African-American or Hispanic), and having certain genetic mutations.
Is there a cure/medications for Dermatopathia pigmentosa reticularis?
At this time, there is no known cure or medications for Dermatopathia pigmentosa reticularis. Treatment is focused on managing the symptoms and preventing further skin damage. This may include avoiding sun exposure, using moisturizers, and using topical steroids to reduce inflammation.