• Acquired Melanocytic Nevi: Develop after birth and are often referred to as “moles”. 

• Dysplastic Nevi (Atypical Moles): These moles have an irregular appearance and may be more likely to develop into melanoma, though most remain benign. 

People with giant congenital melanocytic nevus may have more than one nevus (plural: nevi). The other nevi are often smaller than the giant nevus. Affected individuals may have one or two additional nevi or multiple small nevi that are scattered over the skin; these are known as satellite or disseminated nevi.

Affected individuals may feel anxiety or emotional stress due to the impact the nevus may have on their appearance and their health. Children with giant congenital melanocytic nevus can develop emotional or behavior problems.

Some people with giant congenital melanocytic nevus develop a condition called neurocutaneous melanosis, which is the presence of pigment-producing skin cells (melanocytes) in the tissue that covers the brain and spinal cord. These melanocytes may be spread out or grouped together in clusters. Their growth can cause increased pressure in the brain, leading to headache, vomiting, irritability, seizures, and movement problems. Tumors in the brain may also develop.

Causes

The cause of this condition is not clearly understood, but it is thought to result from a defect in embryologic development during the first 12 weeks of pregnancy. The defect is thought to cause a proliferation of melanocytes, the cells responsible for normal skin color. When melanocytes are produced at an extremely rapid rate, they form in clusters instead of spreading out evenly, resulting in abnormal skin pigmentation in some areas of the body.

Genes can influence a person’s moles. Dysplastic nevus syndrome is a largely hereditary condition that causes a person to have a large quantity of moles (often 100 or more), with some larger than normal or atypical. This often leads to a higher risk of melanoma, a serious type of skin cancer. Dysplastic nevi are more likely than ordinary moles to become cancerous. While dysplastic nevi are common, and many people have a few of these abnormal moles, having more than 50 ordinary moles also increases the risk of developing melanoma.

In the general population, a slight majority of melanomas do not form in existing moles but rather create new growths on the skin. Somewhat surprisingly, this pattern also applies to those with dysplastic nevi. These individuals are at a higher risk of melanoma occurring not only where there is an existing mole but also in areas without moles. Consequently, such persons need regular examinations to check for changes in their moles and to identify any new ones.

Diagnosis

Melanocytic naevi are usually diagnosed clinically by their typical appearance. If there is any doubt about the diagnosis, an expert may be consulted in person or with the help of clinical and dermatoscopic images.

• A naevus changes size, shape, structure or colour
• A new naevus develops in adult life (> 40 years)
• It appears different from the person’s other naevi (a so-called ugly duckling)
• It has ABCD characteristics (Asymmetry, Border irregularity, Colour variation, Diameter > 6 mm)
• It is bleeding, crusted or itchy.

Most skin lesions with these characteristics are actually harmless when evaluated by an expert using dermatoscopy. Short-term digital dermatoscopic imaging may be used in equivocal flat lesions to check for change over time.

Naevi that remain suspicious for melanoma are excised for histopathology (diagnostic biopsy). A partial biopsy is not recommended, as it may miss an area of cancerous change.

 Treatment

Most melanocytic naevi are harmless and can be safely left alone. They may be removed in the following circumstances:

• To exclude cancer
• If a naevus is a nuisance: perhaps irritated by clothing, comb or razor
• Cosmetic reasons: the mole is unsightly.

Surgical techniques include:

• Excision biopsy of a flat or suspicious melanocytic naevus
• Shave biopsy of a protruding melanocytic naevus
• Electrosurgical destruction
• Laser to lessen pigment or remove coarse hair.

At any age, sun protection is important to reduce skin ageing and the risk of skin cancer.

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