Vulvar Melanosis (Vulvar Lentiginosis)
It is also called vulvar lentiginosis or vulvar melanotic macules. These lesions represent 68% of pigmented vulvar lesions in women. They are more commonly found among perimenopausal women with a median age of 40–44 years. The labia minora is the most com
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Sandra Ronger Savle
37.1 Clinical Presentation 37.1.1 Vulvar Melanosis Vulvar melanosis is also called vulvar lentiginosis or vulvar melanotic macules. These lesions represent 68% of pigmented vulvar lesions in women. They are more commonly found among perimenopausal women with a median age of 40–44 years. The labia minora is the most commonly affected site. When they are present in children or young adults, one should consider that they represent systemic dermatoses: –– In Peutz-Jeghers syndrome, the oral mucosa, perianal area, hands, and feet may be involved. The genetic mutation is STK11. In this syndrome one may find gastrointestinal hamartomatous polyps. An increased risk of breast, ovarian, pancreatic, and gastrointestinal cancer has been reported. –– LEOPARD syndrome or multiple lentigines syndrome is associated with the genetic mutations: PTPN11, RAF1, and BRAF. Pigmentation is located also on the face, neck, and trunk. Electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormal
S. Ronger Savle (*) Department of Dermatology and Gynecology, Lyon 1 University and Centre Hospitalier Lyon Sud, Pierre Bénite, Lyon, France
genitalia retardation of growth, and sensorineural deafness may also coexist. –– The Carney complex is a mutation of PRKAR1A. It involves the eyelids, conjunctivae, and oral mucosa. Cardiac myxomas, endocrine tumors, blue nevus, and psammomatous melanotic schwannoma may also coexist. –– The Dowling-Degos disease is associated with reticulated hyperpigmentation, follicular papules, comedo-like lesions, perioral scars, and hypopigmented or erythematous macules. The pigmentation sites are axillae, groin, flexural folds, and neck. A mutation of KRT5 is found. –– Bannayan-Riley-Ruvalcaba is located on the face. It is associated with macrocephaly, intestinal hamartomatous polyposis, lipomas, hemangiomas, intellectual disability, joint hyperextensibility, pectus excavatum, and scoliosis. Laugier-Hunziker syndrome is an acquired disorder involving 10% of the population, characterized by benign macular hyperpigmentation of the oral and genital mucosa, which is associated with longitudinal melanonychia in 50–60% of cases. These lesions are benign melanocytic macules characterized by single or often numerous lesions with a tendency to confluence. They can be variegated in color (light brown, dark brown, slate gray, blue, or black) and with a geographic aspect (Fig. 37.1). They are mostly bilateral but
© Springer International Publishing AG, part of Springer Nature 2019 J. Bornstein (ed.), Vulvar Disease, https://doi.org/10.1007/978-3-319-61621-6_37
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The origin of mucosal melanosis is unclear. The excessive pigmentation could be secondary to a chronic stimulus in the area or due to a defect in the normal transport of melanin to suprabasal keratinocytes [4].
37.1.2 Lentigines, Lentigo They may be sporadic or part of a syndrome. It is the most common pigmented lesions of the vulva and occurs as homogeneous dark brown macules, measuring les
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