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                                      D22.0  Melanocytic nevus of lip




Congenital melanocytic nevus syndrome is characterized by pigmentary skin defects apparent at birth. Most individuals have 1 or more large or giant lesions greater than 20 cm and up to over 60 cm in diameter, which may cover up to 80% of total body area. These lesions may or may not be hairy. Smaller 'satellite' pigmented lesions numbering in the hundreds may also be present all over the body. Congenital melanocytic nevi (CMN) can be associated with malignant melanoma , but the risk appears to be low, ranging from 1 to 2% for all individuals, but rising to 10 to 15% in those with very large nevi (greater than 40 cm). A small subset of patients with CMNS have abnormalities of the central nervous system, known as 'neurocutaneous melanosis' or 'neuromelanosis' , which may be symptomatic. Patients with CMNS also tend to have a characteristic facial appearance, including wide or prominent forehead, periorbital fullness, small short nose with narrow nasal bridge, round face, full cheeks, prominent premaxilla, and everted lower lip.

Blue nevi are characterized by a collection of pigment-producing melanocytes in the dermis. These lesions clinically present as well demarcated cerulean-blue or bluish black colored papules or plaques that usually measure less than 1 cm in diameter. They are typically found on the dorsal surface of the hands and feet or in the head and neck region; however, they are rarely found in the oral cavity. These lesions are usually benign and stable over time. However, malignant melanomas developing in or associated with a blue nevus (which is also called malignant blue nevus) have been only rarely reported. A malignant blue nevus might develop in a common blue or cellular blue nevus, a giant congenital nevus or in a nevus of Ota, or it may be malignant from the start. Malignant blue nevi most commonly are found on the scalp. A malignant blue nevus of the lip has not been previously reported in the medical literature. We report here on a patient with a malignant melanoma associated with a blue nevus of the lip. The malignant melanoma was presumed to have developed from a blue nevus that was present on the upper lip of a 50-year-old male.



Oral melanocytic nevi are benign proliferations of nevus cells in the epithelial layer, the submucosal layer, or both. As such, they are classified as junctional, intramucosal, and compound nevi. Nevi may also be classified as congenital or acquired. Unlike their cutaneous counterparts, oral melanocytic nevi are rare.

A tendency to have multiple melanocytic naevi runs in some families. Sunburn or excessive sun exposure contribute to new moles formation and people with fair skin are more at risk.Small nevi can be removed by simple surgical excision. The nevus is cut out, and the adjacent skin stitched together leaving a small scar. Removal of a large congenital nevus, however, requires replacement of the affected skin.a small blue or bluish-black spot, often called a blue nevus, is usually benign but often mistaken for melanoma. Conversely, a junctional nevus, which develops at the junction of the dermis and epidermis, is potentially cancerous.


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