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General
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- This topic covers common non-melamoma pigmented lesions of vulva
Epidemiology
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Diagnosis
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Differential diagnosis
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- Normal vulvar pigmentation
- When compared to the rest of the body, genital skin is usually more pigmented
- Histologically, it is common to see at least some amount of melanin pigmentation within the basal keratinocytes
- Therefore, it is important to know the baseline pigmentation of the patient, since the presence of basal keratinocytic pigmentation can be misleading, especially in small biopsies
- Acanthosis nigricans (Indian J Dermatol 2011;56:678, Fertil Steril 1993;59:583)
- Clinical presentation:
- Pigmented / brown velvety plaques
- Histologic features:
- Mild epidermal acanthosis, papillomatosis (usually with elongated, delicate papillae), hyperkeratosis, horn-pseudocysts
- Mild increase in basal layer pigmentation
- Genital melanosis (J Reprod Med 1993;38:5, Am J Dermatopathol 1985;7:51)
- Clinical presentation:
- Variably extensive and slowly developing pigmentation
- Histologic features:
- Hyperpigmentation of basal keratinocytes
- Melanocyte density is usually normal or very mildly increased
- Pigment incontinence in superficial dermis may be present
- Mucosal melanotic macules (J Am Acad Dermatol 2000;42:640, J Am Acad Dermatol 2014;70:e81)
- Clinical presentation:
- Dark asymmetrical macules
- Histologic features:
- Hyperpigmentation of basal keratinocytes
- Melanocyte density is usually normal or very mildly increased
- No nested or confluent proliferation of melanocytes
- Post-inflammatory pigmentary alteration (Dermatol Ther 2010;23:449)
- Clinical presentation:
- Slowly increasing pigmentation which is often the result of an inflammatory condition
- The initiating inflammatory disease is frequently asymptomatic
- Histologic features:
- Prominent pigment incontinence with melanin lying free in the superficial dermis or more frequently, increased numbers of pigmented melanophages
- Lichen simplex chronicus (Dermatol Clin 2010;28:669)
- Clinical presentation:
- Dark, pigmented, thickened skin
- Histologic features:
- Epidermal acanthosis (may be slightly irregular), hyperkeratosis (often with presence of stratum lucidum), hypergranulosis
- Fibrosis or presence of thickened, vertically oriented collagen fibers in papillary dermis
- Superficial perivascular lymphohistiocytic infiltrate
- Pigment incontinence may be present
- Pigmented vulvar intraepithelial neoplasia / pigmented bowenoid papulosis (Gynecol Oncol 1980;10:201, An Bras Dermatol 2014;89:825, J Am Acad Dermatol 2010;62:597)
- Clinical presentation:
- Histologic features:
- Usual type vulvar intraepithelial neoplasia (squamous cell carcinoma in situ or Bowen's disease) with full thickness keratinocytic atypia
- Melanin pigment often in small dense clusters within the epithelium
- Pigment incontinence may be present
- A lichenoid lymphohistiocytic inflammatory infiltrate may be present
- Pigmented seborrheic keratosis (Clin Exp Dermatol 2005;30:17)
- Clinical presentation:
- Often multiple
- Dark, brown papules or nodules with a stuck on appearance
- Histologic features:
- Epidermal acanthosis, low papillomatosis, hyperkeratosis and horn pseudocysts
- Pigmented condyloma acuminatum (J Dermatol 2014;41:337, J Dermatol 2012;39:860)
- Clinical presentation:
- Dark warty growths with fleshy or filiform appearance
- Often multiple
- Histologic features:
- Epidermal acanthosis with hyperkeratosis and at least focal parakeratosis, with an undulating surface
- The deep aspect of the lesion is fairly regular and sharply demarcated
- Superficial keratinocytes with perinuclear halo, hyperchromatic nuclei and irregular / wrinkled nuclear contours (koilocytes), consistent with human papilloma viral cytopathic effect
- Pigmented basal cell carcinoma (Dermatol Online J 2011;17:8, Acta Dermatovenerol Alp Pannonica Adriat 2011;20:81)
- Genital melanocytic nevi (Eur J Gynaecol Oncol 2002;23:323, J Cutan Pathol 2008;35:889, Dermatology 2010;221:55, Dermatol Online J 2010;16:9)
- May affect about 2% of women
- Any type of nevus can be seen in the vulva
- Clinical presentation:
- Irregular pigmented lesion up to 1 cm in size
- Lesions typically involve labia minora, labia majora or mucosal aspect of clitoris
- Histologic features:
- Some degree of architectural disorder is allowed in special sites such as vulva
- Nevi may be junctional or compound and may be asymmetrical
- The junctional component is composed frequently of lentiginous proliferation of melanocytes, which is often nested
- The nests may be large with retraction artifact and usually arise from the sides of rete ridges and may involve the suprapapillary plate
- Adnexal extension may be seen
- Dermal melanocytes mature with dermal descent
- The overlying epidermis may be hyperplastic
- Due to frequent irritation, features of trauma such as focal pagetoid spread of melanocytes, pigmented parakeratosis and transepidermal elimination of melanocytic nests may be seen
- Genital dysplastic nevi
- Clinical presentation:
- Irregular pigmented lesion, often on labia majora
- Histologic features:
- Features overlap with atypical genital nevi (J Cutan Pathol 2008;35:24, Am J Surg Pathol 2008;32:51)
- More lentiginous spread and elongation of rete ridges may be seen
- Lamellar eosinophilic fibroplasia of papillary dermis is present
- Variable amounts of lymphohistiocytic inflammatory infiltrate may be present
- Angiokeratoma (Eur J Gynaecol Oncol 2011;32:597, Acta Dermatovenerol Croat 2010;18:271)
- Clinical presentation:
- Histologic features:
- Epidermal hyperkeratosis, acanthosis (often irregular) and low papillomatosis in close proximity to thin-walled dilated blood vessels filled with red blood cells in papillary dermis
- Dowling-Degos disease (Ann Dermatol 2011;23:205)
- Clinical presentation:
- Multiple symmetric pigmented papules
- Histologic features:
- Elongation and prominent pigmentation of epidermal rete ridges
- No increase in melanocyte density
- Suprapapillary plate thinning, pigment incontinence and hyperkeratosis may be present
Case reports
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Clinical images
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Hyperpigmented brownish macules
Angiokeratoma of Fordyce
Micro images
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Dowling-Degos disease
Angiokeratoma of Fordyce
Images courtesy of Dr. Priya Nagarajan:
Normal
Dysplastic nevus with mild atypia
Dysplastic nevus with severe atypia
Mucosal melanotic macule
Mucosal melanotic macule, melanocytic cocktail (HMB45, MART1, tyrosinase)
Additional references
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End of Vulva > Malignant neoplasms > Pigmented lesions
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