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Vulvar lesions: Differential diagnosis of red lesions

Vulvar lesions: Differential diagnosis of red lesions
Literature review current through: Jan 2024.
This topic last updated: Oct 13, 2023.

INTRODUCTION — A wide variety of lesions occurs on the vulva. Some of the disorders causing these lesions are limited to the vulva, while others also involve skin or mucocutaneous membranes elsewhere on the body. This topic provides a morphology-based classification system that can help clinicians with the differential diagnosis of these lesions after performing a history and physical examination. This classification system is also useful for guiding the choice of diagnostic tests and procedures.

This topic will focus on differential diagnosis of red vulvar lesions. Discussions of white, other pigmented, erosive and ulcerative, and skin-colored or yellow lesions are presented in related content.

(See "Vulvar lesions: Differential diagnosis of white lesions".)

(See "Vulvar lesions: Differential diagnosis of pigmented (black, brown, blue) lesions".)

(See "Vulvar lesions: Differential diagnosis of vesicles, bullae, erosions, and ulcers".)

(See "Vulvar lesions: Differential diagnosis of yellow, skin-colored, and edematous lesions".)

The pertinent history, physical examination, and diagnostic tests and procedures used to evaluate women with vulvar lesions are described separately (see "Vulvar lesions: Diagnostic evaluation"). Treatment is also discussed separately in topic reviews on these disorders.

In this topic, when discussing study results, we will use the terms "woman/en" or "patient(s)" as they are used in the studies presented. We encourage the reader to consider the specific counseling and treatment needs of transmasculine and gender-expansive individuals.

MORPHOLOGIC DEFINITIONS FOR MUCOCUTANEOUS VULVAR LESIONS — Mucocutaneous vulvar lesions can be classified using the morphologic definitions described in the table (table 1).

DIAGNOSTIC EVALUATION

Practical tips — Based on the authors' experience, clinicians should be aware of the following points when evaluating dermatologic lesions of the vulva:

Alternate appearance of vulvar lesions – Many skin conditions may appear differently on the vulva than on other parts of the skin as the vulva is so moist and the area is commonly subject to friction.

Concomitant conditions – Concomitant conditions to consider with vulvar lesions include infections, other dermatoses, and neoplasia/cancer. For example, irritant contact dermatitis from the use of caustic soaps or overwashing is commonly seen in individuals with vulvar lichen sclerosus.

Impact of topical therapies – Vulvar conditions may be significantly altered by the use of topical treatments. The authors ask patients about use of prescription, over-the-counter, compounded, and alternative topical preparations.

Role of biopsy – Be prepared to perform a biopsy, especially if a vulvar lesion is atypical, bleeding, the patient is immunocompromised, the lesion is not responding to appropriate treatment, or there is concern for a malignancy [1]. For any lesions with variable or irregular areas, more than one biopsy may be needed. At times, the biopsy results can be inconclusive. If there are ever concerns or questions about the biopsy report, it is important to speak with the pathologist. Sending a clinical photograph to the pathologist can also aid the diagnosis.

Consider possibility of systemic disease – Vulvar lesions can represent diseases that commonly present on the vulva (eg, herpes simplex virus) or systemic diseases that manifest in multiple sites including the vulva (eg, psoriasis). Providers are encouraged to ask patients with vulvar lesions about generalized symptoms and presence of lesions or skin changes on other body parts.

Specialist referral – Patients whose symptoms do not respond, or worsen, despite usual treatment should be referred to a gynecologist, dermatologist, family medicine physician, or other health care providers who specialize in vulvar skin disorders.

How to use this topic — After a history, physical examination, and morphologic classification (table 1) have been performed, the information in this topic can be used to begin a differential diagnosis of the lesion. Diagnostic entities are listed for each morphologic classification and often listed under more than one morphologic classification since many lesions have more than one presentation (morphologic type, color). The complete description of the diagnostic entity is provided only once; for morphologic variants, the entity is noted and linked to its descriptive section.

This topic is an overview of red dermatologic lesions of the vulva. Detailed text specific to each type of vulvar lesion is available separately, and links are provided throughout the text where available. Brief summaries of pertinent history, physical examination, and diagnostic tests and procedures are provided in this topic. Discussions of the presentation, evaluation, and diagnosis of vulvar lesions are described in detail separately.

(See "Vulvar lesions: Diagnostic evaluation".)

(See "Approach to the clinical dermatologic diagnosis".)

(See "Skin biopsy techniques".)

RED MACULES, PATCHES, AND PLAQUES — Red patches are large (>1 cm) areas of color change that are neither elevated nor palpable; plaques are large (>1 cm), flat-topped, palpable lesions (table 1). The differential diagnosis for both types of lesions is similar (table 2).

Common diseases — All lesions below are commonly seen in clinical practice and are presented in approximate descending order of frequency. Frequency may vary based on the patient population and type of clinical practice (eg, gynecology, internal medicine, dermatology, etc).

Candidiasis

Physical examination – Candidiasis typically appears as bright red, sometimes eroded patches and plaques involving varying portions of the vulva (picture 1). In severe cases, the perianal area and the upper inner thighs (picture 2) may also be involved. A slight scale may overlie the red patches and plaques. Small 1 to 2 mm pustules may stud the surface of these patches and plaques, and at the periphery of the main area of involvement, similar satellite red, yellow, or white papules and/or pustules may occur. The pustules break easily, leaving small erosions with peripheral ("collarette") scales. If many pustules are present, the desquamating scale can be prominent (picture 3). When skinfolds are involved, linear fissures commonly occur within the crease.

Vaginal discharge due to vaginal candidiasis is usually, but not always, present. Sometimes, white plaques made up of a mat of candidal mycelia can be found loosely attached to the mucous membranes of the vagina and/or vulvar vestibule.

Symptoms – The lesions may be pruritic or painful (eg, rawness or burning).

Diagnosis – The diagnosis is based on a characteristic history, physical examination, and a potassium hydroxide (KOH) preparation to detect fungal elements (mycelia and spores) in vulvar skin scrapings or on a smear of discharge showing hyphae, pseudohyphae, or budding yeast. Vaginal candidiasis supports the diagnosis. Candida culture for diagnostic uncertainty and speciation should be obtained in cases of poor response to therapy or chronic recurrence [2,3]. A molecular-based test using vaginal swabs collected by clinicians or patients can also be considered for diagnosing the most common fungal as well as bacterial and protozoan causes of vaginitis [4]. (See "Candida vulvovaginitis: Clinical manifestations and diagnosis".)

Atopic dermatitis (eczema) — Atopic dermatitis is characterized by dry skin and severe itching. Patients with generalized atopic dermatitis may have a personal or family history of atopy. These patients have easily irritated skin and are more susceptible to irritant contact dermatitis. They are itchy and can develop chronic itch-scratch cycles resulting in lichen simplex chronicus.

(See "Atopic dermatitis (eczema): Pathogenesis, clinical manifestations, and diagnosis".)

(See "The relationship between IgE and allergic disease", section on 'Atopy'.)

Specific to atopic dermatitis of the vulva:

Physical examination – Findings of contact dermatitis (usually irritant) or lichen simplex chronicus are seen and include red, poorly marginated, excoriated or lichenified plaques predominately involving the labia majora and the outer aspect of the labia minora; the vestibule is generally spared. Weeping and crusting may be seen. Scale is usually present with atopic dermatitis, but when the vulva is involved, the scale is often obscured because moisture may whiten the surface of the patches and plaques.

Symptoms – The predominant symptom is pruritus. Incessant scratching leads to more itching and more scratching, which is termed the "itch-scratch cycle." Development of numerous or deep excoriations may be associated with pain [5]. (See 'Lichen simplex chronicus' below.)

Diagnosis – A diagnosis of atopic dermatitis is usually based on the history of atopy and clinical findings; rarely, patch testing and/or biopsy is necessary to identify an allergen/irritant or exclude an underlying dermatosis. A history of atopy (hay fever, seasonal allergies, and/or asthma) is present in approximately 70 percent of patients and supports the clinical diagnosis. Atopic dermatitis is an important component of the differential diagnosis in patients with vulvar dermatitis as it is often overlooked and treatment is delayed [6,7].

(See "Vulvar dermatitis", section on 'Clinical manifestations'.)

(See "Atopic dermatitis (eczema): Pathogenesis, clinical manifestations, and diagnosis".)

Irritant contact dermatitis — Irritant contact dermatitis is a common condition that is responsible for approximately one-half of patients presenting with chronic vulvar itching [8,9]. This is one of the most common, and most often missed, causes of vulvar problems and often complicates all other vulvar issues. For example, patients that have genital herpes simplex virus or vulvar lichen sclerosus may also have an irritant contact dermatitis. Always ask about all the products the patient is using, such as cleansers, lubricants, and pads, to identify potential irritants (table 3).

The most common cause of this condition is excessive hygiene. The resulting dryness leads to a chapped appearance with minute fissures. Urinary incontinence alone, or with the use of pads, is also a common cause of irritant contact dermatitis. Almost any topical product can be responsible for the development of irritant contact dermatitis. Application of imiquimod or fluorouracil regularly results in erosions superimposed on painful red patches and plaques. Application of liquid nitrogen (for genital warts), cantharidin (for molluscum contagiosum) or bi- or trichloroacetic acid (for genital warts) almost always results in blisters that break down, leaving circular erosions or even ulcers, with bits of lifted scale present at the periphery of the erosion. Typical treatment involves removing the vulvar irritants and symptom management [1]. (See "Irritant contact dermatitis in adults".)

Physical examination – Red patches and plaques similar to those seen in other eczematous disease, such as atopic dermatitis and allergic contact dermatitis, are seen [10] (picture 4 and picture 5). (See 'Atopic dermatitis (eczema)' above.)

However, the erythema may be duskier and may be red-brown in color. The skin may be dry and chapped. Fissuring may be present. Very rarely, large numbers of small vesicles or bullae may erupt from the surface of the plaques. The vesicles break easily, leaving small patches of weeping erosions. This process can be confused with an eczematous candidiasis or impetigo. Contact with extremely harsh products such as bi- or trichloroacetic acid can cause erosions or ulcers. The complete physical examination of patients with suspected irritant contact dermatitis can be found separately. (See "Vulvar dermatitis", section on 'Physical examination'.)

Symptoms – The degree of itching and scratching is more variable than with atopic dermatitis. Most patients complain of irritation and discomfort more than itching. Some patients complain of pain and burning, especially when there are erosions and/or ulcers. (See "Irritant contact dermatitis in adults", section on 'Clinical manifestations'.)

Diagnosis – A diagnosis of irritant contact dermatitis is based on a history of either excessive hygiene or the persistent presence of too much moisture, such as might occur in diaper dermatitis or in bedridden, incontinent adults. Irritant contact dermatitis may also be induced by a patient's application of harsh chemicals (such as cleansing wipes, undiluted bleach, strong detergents, and rubbing alcohol) or a clinician's application of caustic medications (such as podofilox, fluorouracil, imiquimod, and bi- or trichloroacetic acid). (See "Vulvar dermatitis" and "Irritant contact dermatitis in adults", section on 'Diagnosis'.)

If bullae are present (very rare), differential diagnosis includes bullous pemphigoid and pemphigus. Bullous pemphigoid also can have itchy lesions, but bullae are usually unilocular and tense. Pemphigus vesicles and bullae are extremely fragile and are not itchy. (See "Vulvar lesions: Differential diagnosis of vesicles, bullae, erosions, and ulcers", section on 'Pemphigus vulgaris' and "Vulvar lesions: Differential diagnosis of vesicles, bullae, erosions, and ulcers", section on 'Bullous pemphigoid'.)

Lichen simplex chronicus — Lichen simplex chronicus is a reactive skin pattern with thick, indurated skin secondary to chronic, habitual rubbing or scratching. It is most commonly seen on the vulva associated with atopic dermatitis, psoriasis, and contact dermatitis. It can also be seen in chronically scratched vulvar lichen sclerosus and even in conjunction with squamous intraepithelial lesions (SIL). It is a common cause of vulvar pruritus manifesting as nightly scratching. Classically, there are itch-scratch cycles that are uncontrollable. It is frequently seen in health care providers' offices [11].

Physical examination – Lichen simplex chronicus typically presents with a thickened, red epidermis (picture 6). The appearance is similar to atopic dermatitis except the plaques may be a deeper red or a red-brown (picture 7). (See 'Atopic dermatitis (eczema)' above.)

The skin is thick and leathery, typically with an increase in skin markings referred to as lichenification. Palpate these lesions to feel the typically thick lichenification to make the diagnosis. The diagnosis is usually clinical. When chronically exposed to moisture from sweat, urine, or vaginal secretions, the affected skin whitens (picture 8 and picture 9). This phenomenon is similar to the whitening seen over the volar fingertips after swimming for a prolonged period of time [12].

In some circumstances, such as after partial treatment, inflammation is not clinically visible, so the plaques may be skin colored. Lichenification due to chronic rubbing usually predominates, although some degree of erosion and fissuring due to excoriation is regularly present. Excoriation can result in ulcers. Scale is present, but surface roughness may be absent because it is compacted, and it may be obscured because of whitening from chronic moisture.

Symptoms – The same "itch-scratch cycle" is often intense, leading to vigorous scratching and rubbing. The scratching "feels so good"; thus, the patient continues to scratch, resulting in increased itching. These cycles become uncontrollable. Heat, humidity, and sweating exacerbate this condition. Symptoms often worsen at night, and the patient cannot sleep. Anxiety and depression are frequently seen in association with lichen simplex chronicus.

Diagnosis – A diagnosis of lichen simplex chronicus is based on the typical history and physical examination findings above. Rarely, biopsy will be needed. A history of atopy (hay fever and/or asthma) may be present. (See "Vulvar dermatitis".)

Psoriasis — Psoriasis is a common inflammatory skin disease. The epidemiology, clinical manifestations, and diagnosis of psoriasis are reviewed elsewhere. Psoriasis in the skinfolds can be referred to as inverse psoriasis. Psoriasis is often missed on the vulva as most women have never mentioned it to their caregivers despite the fact that 50 to 75 percent of adults with psoriasis have it on their external genitalia. Always ask psoriasis patients if they have genital rashes. (See "Psoriasis: Epidemiology, clinical manifestations, and diagnosis".)

Individuals with vulvar psoriasis typically have the following for presentation and diagnosis:

Physical examination – One or more sharply marginated, scaling, red plaques predominantly involving the labia majora and mons pubis (picture 10). Typically, well-demarcated, scaly plaques in the hairy area are present. These can extend into the skinfolds/creases. In the gluteal cleft, the plaques are red, often fissured, and without any scale. Excoriation and/or lichenification may be encountered. In most instances, more typical psoriatic plaques are also located elsewhere (buttocks, scalp, elbows, knees). Rarely, psoriasis is localized only to the vulva. It commonly involves the crural and gluteal folds [7].

Symptoms – Itching is common. Patients frequently experience sexual dysfunction. At times, there can be significant pain, burning, and dyspareunia.

Diagnosis – A diagnosis of psoriasis can be made clinically if typical psoriatic plaques are located elsewhere on the body. When only the vulva is involved, biopsy is necessary for definitive diagnosis. However, the pathology report may state "psoriatic dermatitis." In such instances, the clinician needs to distinguish psoriasis from lichen simplex chronicus (see 'Lichen simplex chronicus' above), which can be difficult if itching and rubbing are present [13,14].

Dermatoscopy can be helpful for diagnosis. At low magnification (10X), the clinician will see the characteristic "red dots" homogeneously distributed over the lesion on a red background [15]. (See "Psoriasis: Epidemiology, clinical manifestations, and diagnosis", section on 'Diagnosis'.)

Less common diseases

Allergic contact dermatitis — Allergic contact dermatitis of the vulva is not common. Most allergic contact dermatitis of the vulva results from topical medications, soaps, and cosmetics, but the condition can be caused by other sources, such as poison ivy and poison oak [9]. (See "Clinical features and diagnosis of allergic contact dermatitis".)

Physical examination – Allergic contact dermatitis may present as an acute dermatitis with a sudden onset or, most commonly, a chronic, nonresponsive rash. Its appearance as red, somewhat scaly patches and plaques with nonsharp borders is similar to that of atopic dermatitis. (See 'Atopic dermatitis (eczema)' above.)

Rarely, as an acute eruption, there are large numbers of small vesicles, and very rarely, bullae may erupt from the surface of the plaques. The vesicles break easily, leaving small patches of weeping erosions. This process can be confused with an eczematous candidiasis or impetigo. Erosions may also be due to excoriation. The inflammation that is locally present in allergic contact dermatitis may be sufficient to cause appreciable erythema and vulvar edema (picture 4 and picture 5).

Symptoms – The degree of itching and scratching is more variable than with atopic dermatitis. Some patients with a severe eroded allergic reaction complain of burning, pain, or soreness.

Diagnosis – A clinical diagnosis is suggested by the presence of some combination of overlying eczematous features, such as erythema, scale formation, visible erosion, weeping and crusting, and a history of topical medication, soap, or lotion use. A firmer diagnosis of allergic contact dermatitis can be made if discontinuation of all topical agents is followed by clearing of the eruption within 10 days, which helps distinguish this phenomenon from irritant contact dermatitis. (See 'Irritant contact dermatitis' above.)

Biopsy is usually not helpful in separating allergic contact dermatitis from other eczematous diseases unless numerous eosinophils are present within the dermal inflammatory infiltrate. Patch testing to identify the allergen, performed in a dermatologist's or allergist's office, is desirable [16,17]. (See "Vulvar dermatitis" and "Clinical features and diagnosis of allergic contact dermatitis".)

If bullae are present, differential diagnosis includes bullous pemphigoid and pemphigus. Bullous pemphigoid also can have itchy lesions, but bullae are usually unilocular and tense. Pemphigus vesicles and bullae are extremely fragile and are not itchy. (See "Vulvar lesions: Differential diagnosis of vesicles, bullae, erosions, and ulcers", section on 'Pemphigus vulgaris' and "Vulvar lesions: Differential diagnosis of vesicles, bullae, erosions, and ulcers", section on 'Bullous pemphigoid'.)

Lichen planus (papulosquamous form) — The cause of papulosquamous (hypertrophic) lichen planus is unknown, though lesions similar in clinical and histologic appearance can occur in some drug reactions and in graft versus host disease. Detailed discussions of general and vulvar lichen planus are presented elsewhere.

(See "Lichen planus".)

(See "Vulvar lichen planus".)

Briefly, the findings and characteristics of the papulosquamous form of vulvar lichen planus include:

Physical examination – While much less common than the erosive form, papulosquamous lichen planus appears as red or violaceous, sharply marginated, flat-topped papules and/or plaques. Scales are usually not seen. Occasionally, there is evidence of the Koebner phenomenon (the appearance of vitiligo at sites of repeated trauma), and the configuration of the lesions may be linear or angular rather than round. The distribution in the papulosquamous form of lichen planus spares the mucosal surfaces of the vestibule and the vagina, and instead, lesions are found on the labia majora and surrounding cutaneous areas. Extragenital lesions, typically seen on the wrists, ankles, lower legs, arms, and sacral area, are usually found in women with this papulosquamous form of lichen planus. It can mimic other vulvar conditions such as low- or high-grade squamous intraepithelial lesions, or cancer [18].

A lacy (latticelike) network of thin (<1 mm) white or gray lines may be present in the red or red-purple lesions (picture 11). Similar changes, called Wickham striae, may also be present on the buccal surfaces of the mouth (picture 12).

The more common erosive form of lichen planus is discussed separately. (See "Vulvar lesions: Differential diagnosis of vesicles, bullae, erosions, and ulcers", section on 'Erosive lichen planus'.)

Symptoms – In contrast with erosive lichen planus, which is often exquisitely painful, the papulosquamous form is either asymptomatic or somewhat pruritic.

Diagnosis – It is important to look at the rest of the skin for evidence of typical lichen planus. Look for pink to purple papules on the wrists, ankles, and sacrum. While a clinical diagnosis is often possible, we advise making an attempt to confirm the diagnosis with biopsy. The most prevalent histologic finding is a lymphocyte-predominant band of inflammation [19]. As with lichen sclerosus, biopsies to exclude superimposed squamous cell carcinoma (SCC) should be performed for notably thickened or ulcerated areas.

(See "Vulvar lichen planus", section on 'Diagnosis'.)

(See "Vulvar lichen planus", section on 'Papulosquamous lichen planus'.)

Tinea cruris — Detailed discussions of dermatophyte infections are available separately. (See "Dermatophyte (tinea) infections".)

For patients with vulvar tinea cruris, evaluation should include:

Physical examination – Sharply marginated, slightly scaling, dusky red patches or plaques bilaterally on the upper inner thighs (picture 13 and picture 14), possibly extending to the buttocks (picture 15). The labia majora are typically spared. A distinctive feature is the regular presence of clearing (normal-appearing skin) in the center of these patches or plaques, giving an annular appearance. The colloquial term "ringworm" has been used to describe this characteristic finding.

Symptoms – Pruritus, sometimes marked, is usually present, but in some patients, the lesions may be asymptomatic.

Diagnosis – A clinical diagnosis is often possible, but it should be confirmed by scraping some scale from the periphery of the lesion and either submitting it for fungal culture or microscopically examining the scale for hyphae using a KOH wet mount. (See "Dermatophyte (tinea) infections", section on 'Tinea cruris'.)

Perianal streptococcal infection — Perianal streptococcal infection is a bacterial infection usually caused by Group A beta-hemolytic Streptococcus. It is common in children; however, it can occur in adults [20,21].

Physical examination – Bright red patches are seen on the perianal skin (picture 16) and, very rarely, on the vulva. Little scale is present, but minor fissuring, weeping, and crusting may be noted.

Symptoms – The most common symptom is burning. The degree of itching is variable.

Diagnosis – This diagnosis can be considered when other diseases are ruled out and/or when treatments (topical steroids, lubrication, antifungal and anti-candidal agents, etc) used for other diseases have failed to improve or clear the eruption. At that point, bacterial culture demonstrating streptococcal species is necessary. (See "Cellulitis and skin abscess: Epidemiology, microbiology, clinical manifestations, and diagnosis".)

Squamous intraepithelial lesions — Previously termed "vulvar intraepithelial neoplasia (VIN)," high-grade SIL (HSIL) and differentiated VIN (dVIN) are the most common vulvar precancerous lesions [22]. Both HSIL and dVIN may present as red lesions. HSIL are multifocal, human papillomavirus (HPV)-related, and found in younger women. dVIN is not usually HPV-related and is more often unifocal than HSIL. It tends to occur in older women with lichen sclerosus and is less commonly seen in patients with lichen planus [23-27]. By contrast, low-grade SIL, which includes HPV infections and warts, generally presents as white or skin-colored lesions and is not precancerous.

A complete discussion of terminology, presentation, and treatment is presented elsewhere. (See "Vulvar squamous intraepithelial lesions (vulvar intraepithelial neoplasia)".)

Physical examination of patients with vulvar HSIL or dVIN reveals:

HSIL – HSIL is the most common type and is caused by HPV 16, 18, 31, 33, 45 and other rarer, high-risk HPV types. It presents with varying numbers of sharply marginated, flat-topped papules anywhere on the vulva. The lesions may be red, brown, white, or gray-black (picture 17 and picture 18 and picture 19 and picture 20). They may also be skin colored at times. Similar-appearing, larger plaques may be present when the papules have undergone centrifugal or confluent growth. The surface may be hyperkeratotic and/or eroded. Scale is usually not apparent. These lesions tend to occur in younger women but may also be seen in older women.

dVIN – Non-HPV-related dVIN is the less common, but more serious, condition and often presents with a solitary, white to pink, red, skin-colored, or brownish papule or nodule, generally 2 to 5 cm in diameter. However, it can present with one or several red, sharply marginated plaques with varying amounts of overlying scale. Although these lesions are generally not HPV related, they can be similar in appearance to the usual type if they arise de novo from normal-appearing skin. However, more commonly, dVIN develops against a background of other diseases, most often in the setting of lichen sclerosus or, less commonly, in lichen planus. It tends to occur in older women [26].

Symptoms – Both types of vulvar SILs are relatively asymptomatic. Pruritus and/or pain may be noted. (See "Vulvar squamous intraepithelial lesions (vulvar intraepithelial neoplasia)", section on 'Clinical presentation'.)

Diagnosis – A diagnosis of HSIL or dVIN may be suspected based on clinical findings but should be confirmed by biopsy to exclude other conditions such as condylomata acuminata, lichen sclerosus, and invasive SCC. The biopsy should be taken from a notably thickened area or the margin of any chronic erosion or ulcer that fails to respond to medical therapy, especially in the setting of vulvar lichen sclerosus or erosive lichen planus. The biopsy request form should indicate that dVIN is suspected as, without that information, the pathologist may interpret the specimen as a benign acanthotic process such as occurs in psoriasis, psoriatic dermatitis, and lichen simplex chronicus. (See "Vulvar squamous intraepithelial lesions (vulvar intraepithelial neoplasia)", section on 'Biopsy'.)

Rare diseases

Plasma cell vulvitis (Zoon's vulvitis) usually presents as a unilateral, solitary, sharply marginated, deep red to rusty to brownish macules, patches, and petechiae within the vestibule (picture 21 and picture 22) [28,29]. The surface may appear shiny or somewhat eroded. It is nonerosive and nonscarring. There may be a vaginal discharge and associated discomfort from an inflammatory vaginitis. Most lesions are asymptomatic, but itching, pain, or burning may be present. Dyspareunia is common. Biopsy is required for diagnosis. (See "Vulvar lichen planus" and "Vulvar lichen planus", section on 'Differential diagnosis'.)

Extramammary Paget disease presents as one or more usually itchy red patches or plaques on keratinized skin or modified mucous membranes (picture 23). These lesions may be sharply or diffusely marginated with a variably scaly or crusted surface. The surface of the lesions usually demonstrates multiple shallow red erosions often with islands of white, thick epithelium. It looks like psoriasis, lichen simplex chronicus, or contact dermatitis. This disease tends to occur in women over the age of 60 years. Biopsy is required for diagnosis. (See "Vulvar cancer: Epidemiology, diagnosis, histopathology, and treatment", section on 'Paget disease of the vulva'.)

Hailey-Hailey disease [30] and the related papular acantholytic dyskeratosis [31,32] usually present as red patches and plaques, often with varying degrees of fissuring or erosion (picture 24). These lesions mimic the appearance of psoriasis, eczema, tinea cruris, and those described for extramammary Paget disease. However, if there is significant hyperkeratosis, the surface of the lesion may appear white when moist. This disease first appears in younger women, and there are almost always similar plaques on the chest, back, and axillae (table 2) and a positive family history for similar lesions. Biopsy is required for diagnosis [33]. (See "Hailey-Hailey disease (benign familial pemphigus)".)

Tinea versicolor is caused by Malassezia and presents as asymptomatic pink, sharply marginated macules and small patches (picture 25). A fine scale is present but may not be detectable. The vulva is very rarely involved, but when it is, additional pink, red, white, or brown lesions will be found elsewhere, especially over the chest and back. It is a disorder of young women. The diagnosis requires identifying characteristic mycelia and spores in a KOH wet mount of a scraping. (See "Tinea versicolor (pityriasis versicolor)".)

Erythrasma is an infection caused by Corynebacterium minutissimum. It is caused by a Gram-positive aerobic bacterium that favors moist environments such as the vulva. It affects the skin folds. In the vulvar area it presents in the crural and inguinal creases and out onto the inner thighs (picture 26). It presents with brown patches or thin plaques in intertriginous areas. It is seen in conjunction with immunosuppression, diabetes, and obesity.

Physical examination – Skin examination shows a diffuse plaque with erythema or centrally and hyper pigmentation around the edge. The skin is pink to tan colored with well-defined patches or plaques that have a find crinkled surface that fluoresces the typical coral-red color under Wood's lamp (picture 26).

Symptoms – Symptoms of erythrasma consist of itching at times. The areas can be mildly itchy or irritated but are usually asymptomatic.

Diagnosis – A diagnosis can be confirmed by observing a coral-pink fluorescence during Wood's lamp examination of the affected skin (picture 26). Culture is rarely required for this diagnosis. It can also be observed on skin biopsy [34]. (See "Erythrasma".)

Pityriasis rosea mostly affects the skin of the trunk extending, at times, towards the mons pubis. It consists of sharply marginated, oval-shaped, pink patches and plaques with a thin collarette of scale. The vulva is rarely involved, and involvement is always associated with similar oval lesions in a typical "Christmas tree" distribution over the chest and back. Often, there is one initial lesion that is larger than the others called a herald patch. Diagnosis is based on clinical findings but can be confirmed by biopsy, if necessary. (See "Pityriasis rosea".)

Steroid dermatitis – Chronic use of potent or super potent topical corticosteroids (table 4) can result in a steroid/corticosteroid dermatitis (picture 27). The keratinized skin of the vulva, the labia majora, mons, crural folds and inner thighs are particularly involved. The affected areas have ill-defined burning, stinging, and bright red skin with variable telangiectasia. Stopping the topical steroid causes a rebound flair of the redness and symptoms. The topical application must be discontinued [35].

RED PAPULES AND NODULES — Papules are small (<1 cm) palpable lesions while nodules are large (>1 cm) palpable lesions that are usually dome shaped (table 1). The differential diagnosis for both types of lesions is similar (table 5).

Common etiologies

Folliculitis — The pathophysiology of folliculitis may be infectious or noninfectious. Infections are usually due to either a staphylococcal bacterial infection or dermatophyte fungal etiology. Very rarely, Demodex folliculitis is seen. The presentations for these types of infections are similar, although fungal folliculitis almost always occurs in the setting of tinea cruris (ringworm) on the upper inner thighs, whereas bacterial folliculitis occurs de novo as isolated lesions. The evaluation and treatment of bacterial, fungal, viral, and Demodex folliculitis are presented separately. (See "Infectious folliculitis".)

Physical examination – Bacterial and dermatophyte infections manifest as scattered or groups of follicular pustules and small (2 to 5 mm), perifollicular, red papules, most commonly on the hairy areas of the mons pubis, inner thighs, and buttocks, often in shaved areas (picture 28A-D and picture 29). The center surface of the papules may be capped with 1 to 2 mm, white or yellow-white pustules. Erythema may be more difficult to appreciate in patients with darker skin (picture 28C).

Symptoms – Pruritus is the most common symptom associated with folliculitis. Patients may also experience painful pustules.

Diagnosis – The clinical diagnosis of infectious folliculitis can be confirmed by recovery of Staphylococcus aureus on culture, identification of mycelia on a potassium hydroxide (KOH) preparation, or recovery of any one of several dermatophyte fungal species on culture. Demodex can be seen by KOH preparation or biopsy. Noninfectious folliculitis is diagnosed based on clinical appearance after infection is excluded. Rarely, a biopsy is indicated.

Keratosis pilaris

Physical examination – Keratosis pilaris is a noninfectious folliculitis consisting of numerous, closely set, minute, red papules that lack the pustular summit of infectious folliculitis. It usually affects the skin of the buttocks, lateral thighs, and lateral upper arms (picture 30 and picture 31).

Symptoms – Keratosis pilaris is usually asymptomatic.

Diagnosis – The diagnosis of keratosis pilaris is based on physical examination with identification of the typical lesions in the characteristic distribution pattern, but it can be confirmed by biopsy, if necessary. (See "Keratosis pilaris".)

Angiomas — Angiomas are vascular tumors.

Cherry angiomas – Cherry angiomas present as 0.5 to 5.0 mm, smooth, firm, asymptomatic bright to deep red, sometimes purple papules (picture 32 and picture 33). The location is usually on the trunk and proximal limbs and also the mons pubis. They are common, benign neoplasms made up of clustered capillaries and blood vessels. Other names include senile angiomas or "Campbell de Morgan spots." The lesions increase in number with pregnancy. The diagnosis is clinical, based on characteristic appearance. (See "Overview of benign lesions of the skin", section on 'Cherry angioma'.)

Angiokeratomas – Angiokeratomas (also called angiokeratoma of Fordyce) are asymptomatic; dark red, purple, blue, or black; smooth-surfaced; dome-shaped; benign papules (picture 34). The 2 to 8 mm firm papules are made up of dilated superficial blood vessels with hyperkeratosis of the overlying epidermis. Bleeding may occur secondary to trauma. The lesions can be solitary or multiple. They develop between the ages of 20 to 50 years and increase in number in pregnancy. In contrast to cherry angiomas, angiokeratomas are located only on the labia majora; their color is also different: dusky red to purple. (See "Dermoscopic evaluation of skin lesions".)

Hemangiomas – Hemangiomas may present variously as an individual, small, red papule (picture 34 and picture 35); diffuse telangiectatic patches with thicker plaques; or bright red or dusky purple papules that may grow into large nodules over the first nine months of life and then resolve by age 9 or 10. The deeper, cavernous hemangiomas are purple, nodular swellings and can persist into adult life (picture 36). The lesions are vascular malformations that are present at birth or develop in infancy and childhood. They have a predilection for the face and anogenital area but may occur anywhere. Ulceration is the most common complication of hemangiomas (picture 37). Large, segmental lesions, especially in trauma-prone locations, such as the perineum, as shown in the picture (picture 37), are at increased risk for ulceration. (See "Infantile hemangiomas: Epidemiology, pathogenesis, clinical features, and complications" and "Dermoscopic evaluation of skin lesions".)

Furunculosis

Physical examination – A furuncle presents as a deep, walled-off collection of pus typically 2 to 4 cm in diameter and usually associated with a follicle. When not associated with a follicle, the lesion is called an abscess. Lesions begin as a tender, red, firm papule that rapidly enlarges and becomes a deep-seated nodule that lasts for days. The nodule may be capped by a white or yellow-white pustule. It may gradually expand under the skin or point at the surface and rupture purulent material. Rarely, aggregates of infected furuncles form large, deep, red masses, called carbuncles (picture 38). They occur in areas of skin trauma, especially where there is moisture from hyperhidrosis along the edges of the mons pubis and perianal areas. Furuncles and abscesses may be self-limited or recurrent. (See "Cellulitis and skin abscess: Epidemiology, microbiology, clinical manifestations, and diagnosis" and "Cellulitis and skin abscess: Epidemiology, microbiology, clinical manifestations, and diagnosis", section on 'Skin abscess'.)

Symptoms – These are painful lesions. When large and deep, they may also be associated with systemic symptoms of malaise, fever, and chills.

Diagnosis – Furuncles and abscesses are caused mainly by S. aureus, and increasingly, methicillin-resistant S. aureus (MRSA). Diagnosis is based on the clinical presentation and appropriate bacterial cultures, which typically show a single bacterial species (S. aureus). These lesions must be differentiated from an infected epidermal cyst and from hidradenitis suppurativa. Furunculosis will typically respond to appropriate antibiotics in 7 to 10 days, in contrast to the longer lasting hidradenitis suppurativa. (See "Vulvar abscess" and "Hidradenitis suppurativa: Pathogenesis, clinical features, and diagnosis" and "Cellulitis and skin abscess: Epidemiology, microbiology, clinical manifestations, and diagnosis", section on 'Diagnosis'.)

Hidradenitis suppurativa — Hidradenitis suppurativa is a chronic, follicular, inflammatory skin condition that is also referred to as "acne inversus." Detailed topics discussing the diagnosis, management, and surgical treatment of hidradenitis suppurativa are presented separately.

(See "Hidradenitis suppurativa: Pathogenesis, clinical features, and diagnosis".)

(See "Hidradenitis suppurativa: Management".)

(See "Surgical management of hidradenitis suppurativa".)

Individuals with hidradenitis suppurativa typically have the following findings, symptoms, and diagnosis:

Physical examination – This condition is characterized by deep-seated, painful, inflamed papules and nodules that may occur anywhere in the anogenital area, most commonly in the inguinal and labiocrural folds, mons pubis, and perianal area (picture 39 and picture 40). The inflammatory papules and nodules are rarely capped by white or yellow-white pustules (picture 41). In addition, solitary pustules may occur unassociated with inflammatory papules and nodules. (See "Hidradenitis suppurativa: Pathogenesis, clinical features, and diagnosis", section on 'Clinical manifestations'.)

An individual lesion may increase in size, form a deep abscess (picture 42), rupture, and drain purulent material, then resolve and/or recur to form a chronic sinus (picture 43). The lesions are usually associated with old scars that are similar to those that can occur with cystic acne. A very distinctive clinical feature is the presence of classic multiheaded or tombstone comedones ("black heads").

Vulvar edema frequently accompanies the inflammatory lesions when the disease occurs in the axillae, under the breasts, on the lower abdominal wall, the vulva, and/or the perianal area. The edema regularly involves the labia majora but less often involves the labia minora. The edema is skin colored, unless inflammatory lesions involve the vulva or adjacent skin, in which case the edema is erythematous. The edema is soft early on and becomes firmer later. The diagnosis is established when the vulvar edema is accompanied by the typical lesions of hidradenitis suppurativa. With time, edema results in the formation of lymphangiomas that present as cystic, clear, pink or red papules.

Symptoms – The swollen, papular, and nodular lesions are usually very painful, and at times, they can be itchy. Ruptured, draining nodules, especially with sinuses, result in wet, potentially foul-smelling, sometimes bloody, raw, painful areas. Anxiety and depression can be debilitating. Sexual dysfunction and dyspareunia are common.

Diagnosis – The diagnosis of hidradenitis suppurativa is made using three criteria. These are the presence of clinically typical lesions, located in the typical sites, with chronicity. The typical lesions are painful, deep-seated nodules (sometimes referred to as "blind boils"), abscesses, draining sinuses, and tombstone comedones. The typical locations are the axilla, groin, and under the breasts; however, they can also occur on the labia majora and labia minora. These lesions are chronically recurrent lesions for more than six months. All three criteria (lesion type, location, and chronicity) are needed to make the diagnosis. The lesions of hidradenitis suppurativa are differentiated from furuncles and abscesses by bacterial culture (in hidradenitis suppurativa, cultures are usually negative or show only nonpathogenic organisms; however, secondary infection of the lesions does occur). Other distinctive features include a marked tendency for recurrence at the same site and poor response, even to long-term antibiotic therapy. Hidradenitis suppurativa lesions must be differentiated from deep fungal and mycobacterial infections, granuloma inguinale, inflammatory cysts, and Crohn disease (picture 44 and picture 45).

(See "Hidradenitis suppurativa: Pathogenesis, clinical features, and diagnosis", section on 'Diagnosis'.)

(See "Hidradenitis suppurativa: Pathogenesis, clinical features, and diagnosis", section on 'Differential diagnosis'.)

Vestibular papillomatosis — Vulvar papillae are a normal variant of the vulvar anatomy. These are benign [36], asymptomatic, monomorphous, closely set tubular projections 1 to 2 mm that are symmetric, soft, and located in the vulvar vestibule (image 1). They are generally the same color as the surrounding mucosal surfaces but are occasionally redder than the surrounding tissue. They are commonly mistaken for warts (condylomata acuminata), which are firm, often with a cauliflower-type appearance (multiple projections off a single base) and are distributed in a less symmetric fashion. The vestibular papillae have a single base to each papillary projection while condylomata acuminata have multiple projections coming off of one single base. (See "Condylomata acuminata (anogenital warts) in adults: Epidemiology, pathogenesis, clinical features, and diagnosis".)

Less common etiologies

Epidermoid cysts (inflamed) — An epidermal/epidermoid cyst (also called sebaceous cyst) is a keratin-filled, subcutaneous cyst originating from a hair follicle. Epidermoid cysts are the most common cysts on the vulva. They are lined by epithelial cells, and their interior is filled with keratin made by these epithelial cells. These cysts have been reported in association with female genital cutting (circumcision) [37]. (See "Overview of benign lesions of the skin", section on 'Epidermoid cyst'.)

Physical examination – The lesions can be single or multiple. They are generally smooth, mobile, round, firm papules or nodules, generally ranging in size from 2 mm to 2 cm in diameter (picture 46 and picture 47); however, they can be smaller or larger. They occur on the labia majora but usually not in the vulvar vestibule. Extrusion of keratin from the cyst into the adjacent dermis causes foreign body type inflammation, with consequent reddening of the nodule. The initial inflammation is not caused by bacterial infection, although, rarely, secondary infection of an already inflamed cyst occurs.

Small (1 to 3 mm) epidermoid cysts (milia) are usually tiny, white papules, but these also may have a slightly yellow color. Inflamed lesions are slightly pink, skin colored to red (picture 48). Uninflamed lesions are white to yellow-white (table 6). One or two or many lesions may occur, isolated or in groups.

Symptoms – These are usually asymptomatic, presenting as a cosmetic issue. However, at times, they can become sore and irritated, especially when located in areas of trauma from sitting or clothing. Patients purposely squeeze these, which typically worsens symptoms. The odor after rupture can be foul.

Diagnosis – A diagnosis can usually be made clinically. Inflamed lesions (picture 48) can be differentiated from furuncles and carbuncles by the history of a previously noninflamed nodule, the color, the opening to the surface, the cheesy material, and the grouped pattern. Larger epidermal cysts are also included in the differential diagnosis of skin-colored lesions if deep in the tissue (table 7), white lesions if they are more superficial (table 6), and yellow lesions (table 8), particularly in more darkly pigmented patients.

Bartholin duct abscesses — Bartholin duct abscesses are red, painful, fluid-filled, infected, cystic cavities occurring on the vulva (picture 49). The labium minus bisects the middle of the abscess. The abscess may point and drain via one or more tracts. Bartholin duct abscesses are differentiated from other vulvar abscesses, furuncles, and hidradenitis suppurativa by their clinical appearance. They are solitary lesions in the classic location (the 5 or 7 o'clock position on the vestibule, with the labium minus located over the midportion of the cyst) [38]. (See "Bartholin gland masses", section on 'Clinical presentation'.)

Diagnosis is usually possible clinically because of the unilaterality of the swelling and the fact that the labium minus overlies its middle aspect. Additional discussions of clinical presentation, diagnosis, and management are presented separately.

(See "Bartholin gland masses".)

Prurigo nodularis — Prurigo nodularis results from chronic scratching at one or more discrete spots. Persistent scratching and picking result in a thickened, callus-like reaction. The lesions are typically 1.0 to 1.5 cm, pink, red, or brown papules with secondary excoriation and serosanguineous crusting on the surface (picture 50 and picture 51). Scarring can be significant. Prurigo nodularis involving the vulva is not common and, if present, usually develops on the labia majora. The diagnosis is based on a history of chronic scratching and the characteristic clinical presentation. If a biopsy is performed, the results show a nonspecific, acanthotic pattern. (See "Prurigo nodularis".)

Urethral caruncles — Urethral caruncles are single, asymptomatic, bright red papules at the urethral meatus, usually on the posterior lip, in postmenopausal females (picture 52 and picture 53). They may be pedunculated or dome shaped. The surface is friable and bleeds easily, which causes mild hematuria and blood on toilet tissue. The differential diagnosis is urethral prolapse. The presentation and evaluation of urethral caruncles are discussed separately. (See "Urethral caruncle".)

Urethral prolapse — Urethral prolapse is a red, swollen cuff around the urethral meatus that is seen in premenarchal or postmenopausal individuals and has a similar appearance in both patient populations (picture 54) [39]. Dysuria may be present. The lesion is differentiated from the urethral caruncle by its presentation as a circumferential, bright red nodule around the meatus, making a donut shape around the urethral orifice.

Crohn disease — Crohn disease, a full-thickness inflammatory disease most commonly seen in the gastrointestinal system, can affect patients from the oral cavity to the anus. It may present on the vulva (including the perianal area) and can also affect the vagina. Vulvar disease may occur before the typical gastrointestinal disease in 20 to 35 percent of patients [40]. If untreated, vulvar Crohn disease can cause recurrent painful inflammatory nodules with abscesses, aphthous ulcers, fistulas, lymphatic destruction with secondary lymphedema, disfiguring anatomic distortion, and, rarely, squamous cell carcinoma. (See "Clinical manifestations, diagnosis, and prognosis of Crohn disease in adults".)

Physical examination – Red papules and nodules are an uncommon manifestation of Crohn disease, but they can occur. The most common diagnostic lesions include linear (knife-cut) ulcers (especially in the skinfolds), abscesses (which frequently break down to form ulcers), large perianal skin tags, aphthous ulcers, fistulae, and diffuse vulvar edema [41-43].

Knife-cut ulcers – In Western countries, the knife-cut ulcers are the most characteristic lesions and, if present, are pathognomonic for Crohn disease (picture 55 and picture 56). Most often, these anogenital lesions occur after, or at the same time, that a diagnosis of intestinal Crohn disease is made. A review of ulcerative lesions of the vulva is presented separately (table 9). (See "Vulvar lesions: Differential diagnosis of vesicles, bullae, erosions, and ulcers", section on 'Erosions, excoriations, and fissures'.)

Fistulae and/or abscesses – Fistulae and abscesses can be seen anywhere on the vulva or perianal areas in Crohn disease. These are most common perianally (table 10). The openings to the fistulae are often located on the labia majora, surrounded by granulation tissue along the edges, forming slightly friable, red papules or small nodules that may cover the opening to the fistulous track (picture 57 and picture 58 and picture 59). Abscesses may be present as well (picture 60). (See "Perianal Crohn disease".)

The inflammatory nodules in Crohn disease closely mimic the appearance of hidradenitis suppurativa (picture 44 and picture 45). For this reason, the axillae and the breasts should be examined as lesions in these locations suggest that the vulvar lesions are those of hidradenitis suppurativa rather than Crohn disease. Hidradenitis suppurativa and Crohn disease may be seen together at times. (See 'Hidradenitis suppurativa' above.)

Vulvar edema – Vulvar edema occurs in a large percentage of women with Crohn disease, especially when the anus and rectum are involved. Skin-colored or red swelling occurs in both the labia minora and labia majora. Early on, it is soft and pits easily; later, it becomes firmer and pits less easily. Diagnosis is established when edema of the vulva occurs in the setting of established intestinal Crohn disease. The vulvar edema may precede a diagnosis of intestinal Crohn disease. For this reason, a history of gastrointestinal symptoms and possibly a workup for inflammatory bowel disease should be obtained in the presence of what appears to be idiopathic vulvar edema. Edema of the labia minora can cause small areas of outpouching, giving the labium a multilobular or multinodular shape. These labia can be reddish, papular, or nodular. Edema may also result in papules of lymphangiomas with or without inflammation.

The complete physical examination of individuals with suspected Crohn disease is presented separately. (See "Clinical manifestations, diagnosis, and prognosis of Crohn disease in adults", section on 'Physical examination'.)

Symptoms – Vulvar symptoms are variable. There may be no symptoms despite significant swelling and knife-cut ulcers. The abscesses and swollen areas can be sore to severely painful and worsen with pressure from sitting or clothing. Smaller lesions can be itchy. The fistulae will drain and have odor. Any ulcers or erosions can be bloody. Constantly draining, especially foul-smelling areas, is debilitating and, along with the pain, a major cause of anxiety and depression. An associated inflammatory vaginitis causes vulvar burning. Dyspareunia is common. These symptoms may be associated with the cardinal symptoms and signs of Crohn disease that include crampy abdominal pain, diarrhea, fatigue, and weight loss. (See "Clinical manifestations, diagnosis, and prognosis of Crohn disease in adults", section on 'Patient presentation'.)

Diagnosis – A clinical diagnosis of Crohn disease can be confirmed by biopsy of vulvar abnormalities, which may show granulomatous inflammation (noncaseating granulomas) suggestive of Crohn disease. However, the typical granulomas in anogenital lesions are only seen at the time of biopsy in 50 percent of cases. The diagnosis often needs to be made on the clinical appearance of vulvar swelling, the typical ulcers (knife-cut and aphthous ulcers), and perianal disease (perianal tags, swelling, and fissures). A fecal calprotectin level is recommended. This has an 87 percent specificity and 99 percent sensitivity for inflammatory bowel disease. Note that vulvar Crohn disease can precede gastrointestinal Crohn disease by three to four years. (See "Approach to functional gastrointestinal symptoms in adults with inflammatory bowel disease", section on 'Evaluation'.)

(See "Clinical manifestations, diagnosis, and prognosis of Crohn disease in adults", section on 'Diagnostic evaluation'.)

(See "Clinical manifestations, diagnosis, and prognosis of Crohn disease in adults", section on 'Differential diagnosis'.)

Squamous cell carcinoma — Squamous cell carcinoma (SCC) is the most common vulvar malignancy. It may occur alone, but in 30 to 40 percent of cases, it is associated with underlying lichen sclerosus or, less commonly, lichen planus (picture 61). Invasive SCC may be associated with differentiated vulvar intraepithelial neoplasia (dVIN) and with human papillomaviruses (HPV) [25,33,44,45]. (See 'Squamous intraepithelial lesions' above and "Vulvar cancer: Epidemiology, diagnosis, histopathology, and treatment", section on 'Risk factors and etiology'.)

Physical examination – The lesions of SCC are firm, red, white, or skin-colored papules, nodules, or plaques. The surface is often friable. Varying degrees of erosion or ulceration may occur [46]. This is most common with the late nodular stage of SCC, such as malignancies that develop in lichen sclerosus and, less often, in lichen planus. The same phenomenon occurs less frequently in the HPV-related type of SCC. It is important to palpate (touch) the vulva. At times a firm area for a biopsy is located, which assists in diagnosing cancer.

The clinical appearance of SCC is similar to that for high-grade squamous intraepithelial lesions (HSIL) of the vulva or dVIN. (See 'Squamous intraepithelial lesions' above.)

Symptoms – Invasive SCC may be asymptomatic, painful, or very itchy. (See "Vulvar cancer: Epidemiology, diagnosis, histopathology, and treatment", section on 'Clinical presentation'.)

Diagnosis – Diagnosis requires biopsy, as differentiating SCC from HSIL of the vulva visually may be difficult. Separate identification of HPV-related carcinoma from non-HPV-related carcinoma is helpful since the prognosis is generally better with the former type of malignancy. The biopsy request form should indicate SCC as a suspected diagnosis as, without that information, the pathologist may interpret the specimen as a benign acanthotic process. (See "Vulvar cancer: Epidemiology, diagnosis, histopathology, and treatment" and "Vulvar squamous intraepithelial lesions (vulvar intraepithelial neoplasia)".)

Molluscum contagiosum — Molluscum contagiosum is a DNA poxvirus infection that can present anywhere on the skin of children, but when it occurs in adults, it is usually located only in the anogenital area, mons pubis, and inner thighs. (See "Molluscum contagiosum".)

Physical examination – These lesions are firm, smooth, skin-colored, white, and red (when inflamed), dome-shaped papules that are 2 to 3 mm in diameter. Typically, the lesions have a central umbilication with a white core (molluscum body) (picture 62). When scratched, the lesions are crusted and inflamed. There may be a secondary dermatitis around the lesions from scratching.

Symptoms – Mild to significant itching may be present. Children often pick and scratch at these making the lesions swollen, crusted, and sore. (See "Molluscum contagiosum", section on 'Clinical features'.)

Diagnosis – Diagnosis is typically made by the characteristic appearance (picture 62). The lesions can also be scraped with a curette; when this material is flattened on a microscope slide, a Giemsa stain will show the pathognomonic inclusion bodies made of viral protein. Alternatively, a shave biopsy can be used to confirm the diagnosis. These findings differentiate molluscum contagiosum from condylomata acuminata, nevi, basal cell carcinoma, and the lesions of herpes simplex virus infection. Lymphangiectatic lesions (superficial lymphangiomas) are firm, more cystic looking, and yield clear fluid when pierced with a needle.

(See "Molluscum contagiosum", section on 'Diagnosis'.)

(See "Molluscum contagiosum", section on 'Differential diagnosis'.)

Insect bites — Bites from any insect can cause red, inflamed, itchy or sore papules or nodules on the vulva. Bites may be individually scattered over the mons pubis or buttocks, sometimes along the panty line in the case of chiggers, or in groups as from fleas. Scabies can present as red papules and nodules in the vulvar area and buttocks in infants and the institutionalized older adult population (picture 63). Similar lesions arise with an infestation from Phthirus pubis/pediculosis. Careful inspection reveals the 1 to 2 mm crab louse attached to the base of the hairs and small gray to tan nits (eggs) on hair shafts. Insect bites can cause a significant reaction in a person who has been previously sensitized. Secondary changes from scratching are often seen. History and clinical pattern suggest the diagnosis. A biopsy demonstrating an eosinophil infiltrate adds to the evidence supporting the diagnosis. (See "Insect and other arthropod bites".)

Rare etiologies

Pyogenic granuloma – Pyogenic granuloma is a reactive, vascular neoplasm seldom seen on the vulva and consisting of very red, friable, often bleeding papules or nodules on a pinched-in base. Differentiation from an amelanotic malignant melanoma is important. Shave excision is advised for diagnosis. (See "Pyogenic granuloma (lobular capillary hemangioma)".)

Pyoderma gangrenosum – Pyoderma gangrenosum is an uncommon neutrophilic dermatosis that presents as an inflammatory and ulcerative disorder of the skin (picture 64A-D) and may develop on the vulva. While ulceration is typically the most prominent feature, red lesions may be part of the early presentation. (See "Pyoderma gangrenosum: Pathogenesis, clinical features, and diagnosis".)

Kaposi sarcoma – Kaposi sarcoma is exceedingly rare on the vulva. It can present with dusky red to purple papules in a patient with HIV/AIDS. (See "Classic Kaposi sarcoma: Clinical features, staging, diagnosis, and treatment".)

Hidradenoma papilliferum – Hidradenoma papilliferum (also known as mammary-like gland adenoma) is a benign, solitary, 0.25 to 1.00 cm, pink to red papule that can be solid or cystic. It occurs in middle-aged women. A biopsy will confirm the diagnosis [47,48].

Endometriosis – Endometriosis uncommonly occurs on the vulva [49]. It appears as a 1 to 2 cm red or pink papule or nodule, but may be skin colored, blue, or purplish [50]. The lesions can fluctuate with menstrual periods. (See "Endometriosis in adults: Pathogenesis, epidemiology, and clinical impact".)

Pseudowart – Pseudowarts are pink to red, warty-looking papules or nodules with thick, scaly surfaces that are sometimes macerated or eroded. These callused skin papules, often forming in groups of lesions, develop from chronic irritation from diapers in babies or the incontinent older adult. A history of urinary incontinence is important to differentiate these lesions from warts and prurigo nodularis. (See "Prurigo nodularis".)

Langerhans cell histiocytosis (LCH) – LCH presents in adults as red papules or nodules in the skinfolds, with or without areas of erosion and ulceration. It is a rare condition [51]. Prognosis of LCH confined to the gynecologic tract appears to be favorable [51,52]. (See "Clinical manifestations, pathologic features, and diagnosis of Langerhans cell histiocytosis".)

Sarcoidosis – Sarcoidosis rarely involves the vulva, but when present, it appears as smooth-surfaced, skin-colored to red/brownish papules and nodules. This disease is not well understood [53,54]. (See "Overview of extrapulmonary manifestations of sarcoidosis".)

Amelanotic melanoma – Amelanotic melanoma is a rare form of malignant melanoma that can present as a skin-colored, pink, reddish, firm papule or nodule on the vulva. Biopsies of the lesion are usually diagnostic. (See "Locoregional mucosal melanoma: Epidemiology, clinical diagnosis, and treatment", section on 'Vulvovaginal melanoma'.)

Nonobstetric vulvar hematomas – These can occur following blunt trauma and may become large at times. The incidence is very low. It is important to ensure that the hematoma is localized and not affecting other pelvic structures.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Vulvar dermatitis".)

SUMMARY AND RECOMMENDATIONS

Initial evaluation – For individuals with vulvar lesions, evaluation begins with a history, physical examination, and morphologic classification of the lesion (table 1), which allows formation of the differential diagnosis. (See 'Morphologic definitions for mucocutaneous vulvar lesions' above.)

Clinical points – Based on the authors' experience, clinicians should be aware of alternate appearances of vulvar lesions, concomitant conditions, topical therapies, role of biopsy, and possibility of systemic disease when evaluating dermatologic lesions of the vulva (see 'Practical tips' above).

Patients whose symptoms do not respond, or worsen, despite usual treatment should be referred to a gynecologist, dermatologist, family medicine physician, or other health care providers who specialize in vulvar skin disorders.

Red patches and plaques – Red patches are large (>1 cm) areas of color change that are neither elevated nor palpable; red plaques are large (>1 cm), flat-topped, palpable lesions. The differential diagnosis for both types of lesions is similar (table 2). (See 'Red macules, patches, and plaques' above.)

Common – Common causes include candidiasis, atopic dermatitis (eczema), irritant contact dermatitis, lichen simplex chronicus, and psoriasis. These entities are typically diagnosed with a combination of patient history and physical examination. (See 'Common diseases' above.)

Less common – Less common causes include allergic contact dermatitis, lichen planus (papulosquamous form), tinea cruris, perianal streptococcal infection, and squamous intraepithelial lesions. (See 'Less common diseases' above.)

Rare – Rare causes include plasma cell vulvitis, extramammary Paget disease, Hailey-Hailey disease, tinea versicolor, and pityriasis rosea. Diagnosis of these entities typically requires a biopsy. (See 'Rare diseases' above.)

Red papules and nodules – Papules are small (<1 cm) palpable lesions while nodules are large (>1 cm) palpable lesions that are usually dome shaped. The differential diagnosis for both red papules and nodules is similar (table 5). (See 'Red papules and nodules' above.)

Common – Common causes include folliculitis, keratosis pilaris, angiomas (cherry angiomas, angiokeratomas, and hemangiomas), furunculosis, hidradenitis suppurativa, and vestibular papillomatosis. These entities are typically diagnosed with a combination of patient history and physical examination. (See 'Common etiologies' above.)

Less common – Less common causes include epidermoid cysts (particularly when inflamed), Bartholin duct abscesses, prurigo nodularis, urethral caruncles, urethral prolapse, Crohn disease, squamous cell carcinoma, molluscum contagiosum, and insect bites. (See 'Less common etiologies' above.)

Rare – Rare etiologies include pyogenic granuloma, Kaposi sarcoma, hidradenoma papilliferum, endometriosis, pseudowart, Langerhans cell histiocytosis, sarcoidosis, and amelanotic melanoma. A shave excision or biopsy is typically required to confirm these diagnoses. (See 'Rare etiologies' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Drs. T Minsue Chen, Aileen Langston, and Peter Lynch, who contributed to earlier versions of this topic review.

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Topic 128406 Version 14.0

References

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