What are the other Names for this Condition? (Also known as/Synonyms)

• Benign Mucosal Pigmentation of the Vulva
• Melanotic Macules of Vulva

What is Vulvar Melanotic Macules? (Definition/Background Information)

• Melanotic macules are dark spots that may be observed on the skin, mucus membranes of the mouth, genitalia, and in the nails. Melanotic macules are benign and no malignant transformation to a melanoma has been recorded
• A macule indicates the presence of a flat, distinct, discolored area of skin less than 1 cm wide. A macule usually does not involve any change in the thickness or texture of the affected skin
• Vulvar Melanotic Macules are observed on or around the vulva in girls or women; the vulva is the area around the external vaginal opening
• The presence of numerous melanotic macules on the body may be associated with a variety of syndromes including Carney complex, Peutz-Jeghers syndrome, and LEOPARD syndrome. In such cases, the cause of the condition is genetically-influenced
• In most cases, Melanotic Macules of Vulva are asymptomatic and do not present any significant signs and symptoms. No complications are typically associated with these lesions; although in some cases, the macules may be confused for melanoma in situ (a precancerous skin condition)
• In a majority, no treatment of Vulvar Melanotic Macules may be necessary and the healthcare provider may undertake a ‘wait and watch’ approach. The prognosis is excellent with appropriate treatment. However, the overall prognosis may depend upon the severity of the signs and symptoms of any underlying disorder (if any present)

Who gets Vulvar Melanotic Macules? (Age and Sex Distribution)

• Vulvar Melanotic Macules may be present in females of all ages (both children and adults)
• There is no racial or ethnic predilection for this skin condition

What are the Risk Factors for Vulvar Melanotic Macules? (Predisposing Factors)

Presently, there are no risk factors identified for solitary Vulvar Melanotic Macule. In the case of multiple melanotic macules, the following rare genetic conditions may be involved:

• Carney complex
• LEOPARD syndrome
• Peutz-Jeghers syndrome
• LAMB syndrome
• NAME syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Vulvar Melanotic Macules? (Etiology)

The exact cause of Vulvar Melanotic Macules is not well-understood. 

• When numerous macules are observed, they may be found in association with certain genetic conditions or syndromes
• When these macules occur in isolation, they are usually sporadic and no associated conditions are observed

The skin condition is not infectious and does not spread from one individual to another through sexual contact.

What are the Signs and Symptoms of Vulvar Melanotic Macules?

The clinical signs and symptoms of Vulvar Melanotic Macules include the following:

• The presence of pigmented skin lesions/spots on the vulva; mostly on the labia minor, and sometimes on the labia majora
• The melanotic macules may be present at other locations within the female genital tract
• They form discolored flat and asymmetric patches on the vulva that are about 1-5 cm in size
• The macules are pigmented blue-black or may take on dark brown shades; the borders are usually irregular
• The macules may be single or multiple; multiple macules are observed in over half the cases
• The underlying skin or mucosal surface usually remains unaffected; no change in the thickness or texture of the skin is typically observed
• The macules may be present unchanged for many years and some are indistinguishable from a melanoma (a skin cancer type)

How is Vulvar Melanotic Macules Diagnosed?

The diagnosis of Vulvar Melanotic Macules may involve the following procedures:

• The diagnosis is usually made by a complete physical (pelvic) examination and evaluation of medical history and family history
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. A skin biopsy is performed to rule out other similar conditions

Note: A skin biopsy is not usually necessary, but may be performed if the healthcare provider suspects a malignancy such as a melanoma in situ.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Vulvar Melanotic Macules?

The complications of Vulvar Melanotic Macules may include the following:

• The longstanding skin patches can cause emotional stress and cosmetic concerns in some individuals
• The appearance of the pigmented macules may induce a fear of melanoma (which is a type of skin cancer)
• Complications that arise from an underlying genetic condition/syndrome

How is Vulvar Melanotic Macules Treated?

The treatment of Vulvar Melanotic Macules may include the following:

• The healthcare provider may choose to regularly observe the benign lesions; a “wait and watch” approach may be followed, once a diagnosis of a melanotic macule is established. In such cases, no treatment is generally required
• If required, a surgical excision and complete removal of the macule, primarily to address cosmetic issues may be undertaken
• Treatment of Vulvar Melanotic Macules with cryosurgery, intense pulsed light (IPL), or laser surgery can be effective
• Treatment of the underlying associated genetic disorder, if any
• Follow-up care with regular screening and check-ups are encouraged

How can Vulvar Melanotic Macules be Prevented?

There is currently no effective means of prevention of Vulvar Melanotic Macules, because the cause of this condition is unknown.

What is the Prognosis of Vulvar Melanotic Macules? (Outcomes/Resolutions)

• The prognosis of Vulvar Melanotic Macules is typically excellent with or without adequate treatment. No malignant transformations of these macules have been recorded
• If these lesions are associated with any genetic disorders, then the overall prognosis depends upon the severity of the signs and symptoms of the disorder

Additional and Relevant Useful Information for Vulvar Melanotic Macules:

• Carney complex comprises a combination of medical conditions that includes multiple tumors, such as myxomas, schwannomas, endocrine disorders, and pigmented skin conditions

Please read more on Carney complex by visiting the following link below:

https://www.dovemed.com/diseases-conditions/carney-complex-cnc/