Congenital Melanocytic Nevi - Neonatal Disorders


What are Congenital Melanocytic Nevi?

Congenital melanocytic nevi (CMN) are pigmented skin lesions that are present at birth or become evident within the first few weeks of life. These lesions are composed of nevomelanocytes, which are a type of melanocyte. They can range in size from small to giant and can appear anywhere on the body.

How Common are Congenital Melanocytic Nevi?

CMN are relatively rare, occurring in approximately 1 in 100 live births. The prevalence of large or giant CMN is even lower, making them a unique subset of neonatal disorders. Most CMN are small, with giant CMN being much less common.

What Causes Congenital Melanocytic Nevi?

The exact etiology of CMN is not fully understood. They are believed to result from a disruption in the normal migration of melanocytes from the neural crest to the skin during fetal development. Genetic mutations, particularly in the NRAS and BRAF genes, have been implicated in some cases.

What are the Clinical Features?

CMN can vary greatly in appearance. They are usually darker than the surrounding skin and can be flat or raised. The surface may be smooth, nodular, or rough. The size of the nevus is an important clinical feature, as it can range from a few millimeters to several centimeters. Giant CMN can cover large areas of the body and may be associated with hypertrichosis (increased hair growth).

What are the Potential Complications?

The primary concern with CMN, particularly large or giant ones, is the potential for malignant transformation into melanoma. The risk of melanoma is higher in larger nevi, although the overall risk remains low. Other complications may include psychological impact due to cosmetic appearance, and in some cases, physical limitations if the nevus covers joints or other mobility-critical areas.

How are Congenital Melanocytic Nevi Diagnosed?

Diagnosis is primarily clinical, based on the characteristic appearance of the lesion. In some cases, dermoscopy may be used to examine the nevus in more detail. Biopsy and histopathological examination are generally reserved for atypical or suspicious lesions to rule out malignancy.

What are the Treatment Options?

The management of CMN depends on the size, location, and potential for malignancy. For small nevi, monitoring with regular dermatologic examinations may be sufficient. Larger or cosmetically concerning nevi may be treated with surgical excision, laser therapy, or other dermatologic procedures. In cases where surgical removal is not feasible, regular monitoring and patient education about the signs of malignant transformation are essential.

What is the Prognosis?

The prognosis for infants with CMN is generally good, especially for those with small nevi. The risk of melanoma is a significant concern for larger or giant CMN, but with appropriate monitoring and management, many patients can lead healthy lives. Early intervention and regular follow-up are key to managing potential complications.

Can Congenital Melanocytic Nevi be Prevented?

Currently, there are no known methods to prevent the development of CMN. They are thought to arise from genetic and developmental factors that cannot be modified. Research is ongoing to better understand the genetic underpinnings of these lesions, which may eventually lead to preventive strategies or improved treatments.



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Issue Release: 2024

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