What is dysplastic nevus syndrome?
Dysplastic nevus syndrome (DNS), also known as atypical nevus syndrome or familial atypical multiple mole-melanoma syndrome, is an inherited condition caracterized by the presence of multiple atypical nevi (moles) that have an increased risk of developing into melanoma, a type of skin cancer.
1. Genetic Predisposition: DNS is caused by genetic mutations, primarily in the CDKN2A gene. This gene plays a crucial role in regulating cell growth and preventing uncontrolled cell division. Mutations in the CDKN2A gene increase the risk of developing atypical nevi and melanoma. DNS is inherited in an autosomal dominant manner, meaning that only one copy of the mutated gene from either parent is enough to cause the condition.
2. Multiple Atypical Nevi: Individuals with DNS typically develop a large number of atypical nevi. These nevi are characterized by their irregular shape, uneven borders, and variation in color, often ranging from light brown to dark brown or even black. They can be larger in size compared to typical nevi and may have a "fried egg" appearance with a darker center and a lighter surrounding area.
3. Increased Melanoma Risk: The most significant concern in DNS is the increased risk of developing melanoma. People with DNS have a much higher chance of developing melanoma than those without the condition. Melanoma is the most serious type of skin cancer and can be life-threatening if not detected and treated early.
4. Sun Sensitivity: Individuals with DNS are often more sensitive to the sun's ultraviolet (UV) radiation. This sensitivity makes them more prone to sunburn and increases their risk of developing skin cancer. It's essential for people with DNS to practice sun protection measures, including using sunscreen, wearing protective clothing, and avoiding excessive sun exposure.
5. Family History: DNS tends to run in families, and individuals with a family history of the condition or a personal history of multiple atypical nevi should consider undergoing genetic testing to determine if they carry the CDKN2A mutation. Genetic counseling can be helpful in understanding the inheritance pattern and assessing the risk of developing melanoma.
Management of DNS involves regular skin self-examinations, monitoring of atypical nevi, and performing full-body skin exams by a dermatologist. Individuals with DNS should be vigilant about seeking medical attention if they notice any changes in the appearance, size, or shape of their nevi. Early detection and treatment of melanoma can significantly improve the chances of a successful outcome.