CHAPTER 1
Vulvar Dystrophies
Denniz Zolnoun, MD
Department of Obstetrics and Gynecology,
University of North Carolina School of Medicine, Chapel Hill, NC, USA
Pathology Notes: Chad Livasy, MD
Associate Professor, Department of Pathology and Laboratory Medicine, University of North Carolina School of Medicine, Chapel Hill, NC, USA
Background
Vulvo-vaginal symptoms are among the most common reasons that women seek health care; upward of 6 million physician office visits are made by women of all age ranges for vulvo-vaginal symptoms.1 Despite such staggering statistics, most clinicians are not adequately prepared to diagnose and treat chronic vulvo-vaginal symptoms. Overlapping clinical appearance, symptoms, and pathophysiology, compounded by nonspecific histology on biopsy, are the main causes for confusion.
This chapter will focus on the following six nonmalignant vulvo-vaginal conditions that clinically may raise concern about premalignant processes:
- Lichen sclerosis
- Contact dermatitis
- Lichen simplex chronicus
- Lichen planus
- Plasma cell vulvitis
- Desquamative inflammatory vaginitis
These conditions share overlapping symptoms of itching and burning to variable degrees. Collectively, these conditions are challenging to care for due to lack of consensus on diagnosis and treatment, intractable and fluctuating clinical course, overlapping morphology and histology, and significant individual variation in treatment response. Additionally, many of these conditions often coexist, posing yet another layer of complexity in deciphering the cause of a patientās chief complaint. Given the intertwined pathophysiology, it is no wonder that the care of these patients seems more a proverbial shot in the dark than a stepwise methodical process.
The diagnostic definition of these six conditions is based on a constellation of symptoms, morphology, and histopathology. As noted in Table 1.1, the primary complaint of the first three conditions is itching, while the primary complaints of the last three are burning, rawness, and pain with intercourse. Thus, using a symptom-based approach, the discussion of these disorders is divided into two parts: conditions with primary complaints of itching (lichen sclerosis, contact dermatitis, and lichen simplex chronicus), and those with primary complaints of burning/rawness sensation (lichen planus, plasma cell vulvitis, and desquamative inflammatory vaginitis). Vulvar intraepithelial dysplasia (VIN), which is often associated with unilateral and focal itching, will be discussed in Chapter 2.
Lichen sclerosis, contact dermatitis, and lichen simplex chronicus
Lichen sclerosis
Generally, lichen sclerosis (LS), also known as lichen sclerosis et atrophicus, affects women in two extremes of reproductive life: pre-puberty and menopause.2 While the prevalence of LS is unknown in the general population, it is estimated to be as high as 1% and constitutes the most common anogenital dermatitis.3 While most cases of LS appear de novo, LS lesions may develop at a site of injury and traumatized skin (Kobner pheonomenon).3 The most common symptom of LS is intractable itching with secondary burning and rawness from self-inflicted trauma (scratching and rubbing). In addition, it is common for chronic sufferers to develop superimposed contact dermatitis.3ā5 Characteristic findings on physical examination are hypopigmentation and thin wrinkled skin with atrophy of subcutaneous tissue.4 In addition, there is loss of vulvar topography caused by agglutination of the clitoral hood, with the clitorial glans āburiedā under the fused tissue, and flattening of the labia minora. Age distribution and posttraumatic development of LS suggests a hormonal etiology, but to date no association between estrogen metabolism and LS has been identified. Nevertheless, estrogen therapy for associated atrophy is a common practice.
LS is one of the few dermatoses with specific histopathology.6 Lichenoid inflammation (a bandlike upper dermal lymphocytic infiltrate) and dermal homogenization (loss of collagen) together are classic findings in LS. Although the presence of lichenoid inflammation and epidermal basal layer damage is not itself pathoneumonic of LS, this finding in association with dermal homogenization is used by dermatopathologists to render a diagnosis of LS.6
Complications associated with lichen sclerosis
Due to the severe itching associated with lichen sclerosis and the subsequent itch-scratch cycle, patients may develop superimposed contact dermatitis either caused by allergic reaction to a variety of topical agents or by irritation from rubbing. Intractable itching and scratching (the itch-scratch cycle) in turn gives rise to the characteristic skin changes seen with lichen simplex chronicus, namely a thick, leathery, excoriated skin. Comorbid lichen plans and malignancy has also been described in long-standing lichen sclerosis (see Clinical Scenario 1).
Contact dermatitis
Vulvar contact dermatitis is the most commonly overlooked vulvar condition, with a reported incidence of 20% to 30% in specialty vulvar clinics.7Although it is usually not the primary cause of vulvar symptoms, it is often a compounding factor in patients complaining of persistent vulvar pruritus (eg, primary lichen simplex chronicus), irritation (eg, plasma cell vulvitis), or burning (eg, generalized vulvodynia). This is not surprising considering the host of behavioral factors (eg, overzealous hygienic practices and self-medication) and clinical factors (eg, chronic use of high-potency steroids and polypharmacy) that are both associated with intractable vulvo-vaginal symptoms.7,8
Similar to lichen sclerosis, the primary complaint of contact dermatitis is itching.7,9 An associated stinging sensation is common with allergic contact dermatitis, while an associated raw or chafing sensation is suggestive of irritant contact dermatitis (eg, diaper rash). The physical exam findings of contact dermatitis demonstrate varying degrees of redness interposed with normal skin. Although scaly skin is common with contact dermatitis in other areas of the body, it is not usually seen on the vulva.10 The moist and warm environment of the vulvar region does not readily reveal scaly dermatosis; however, with progressive airing one begins to see a scaly dusky hue over the labia, which is suggestive of atopic dry skin.
A cotton swab test is a useful tool for the differential diagnoses of vulvovaginal conditions such as contact dermatitis. The cotton swab test is conducted by applying the tip of the cotton swab perpendicular to the vulvar skin and asking the patient to rate the sensation as āa cotton swab sensationā versus āa pinprick sensation.ā If the application of the cotton swab is perceived as a pinprick, then the test is abnormal (punctuate allodynia), which is indicative of an intrinsic inflammatory process of the skin, such as lichen planus. Patients with contact dermatitis, however, have a normal cotton swab test but demonstrate hypersensitivity to gentle stroking (mechanical allodynia).
Vulvar contact dermatitis can be broadly subclassified into two categories: irritant and allergic.7Both variants of contact dermatitis have similar clinical appearances and often coexist. However, it is the nuances of the clinical presentation that favor one subtype over the other. The hallmark of allergic contact dermatitis in the acute phase is severe pruritus, vesiculation, and most importantly, a tendency to spread beyond the initial site of contact. Biopsy is only of value in ruling out malignant processes (squamous cell carcinoma)5 or premalignant processes (VIN lesions). More often than not, the biopsy results from cases of contact dermatitis are clinically vague, with descriptions such as āchronic inflammation with neurophilic infiltrate,ā āspongiosis,ā āacanthosis,ā and āparakeratosis.ā These descriptors are simply histopathologic correlates of what is observed by the clinician. For example, the clinical correlates of acanthosis are an orange peel-appearing, thick skin, while the correlates of parakaratosis are scaliness and an ashy appearance following exposure to air.
Lichen simplex chronicus
Lichen simplex chronicus (LSC) is divided into two clinical subtypes: primary and secondary.11 Primary LSC refers to a condition that arises de novo on ānormalā skin. As suggested by its alternate name, neurodermatitis,11 primary LCS is commonly associated with anxiety disorders. In contrast, secondary LCS develops because of a preexisting dermatologic disorder such as lichen sclerosis. Although the exact prevalence of LSC is unknown, it is estimated to affect up to 0.5% of the general population in western countries.11 In response to chronic excoriation associated with LCS, the vulvar skin thickens. It can be likened to a callous, similar to what is observed in the extremities. Histologic correlates of this thickening are described in terms of dermal (acanthosis) and epidermal (hyperkeratosis) thickening; otherwise, histologic findings in LSC are nonspecific (Table 1.1).11 In the presence of moisture, the skin assumes a wrinkly, white appearance, similar to how fingertips will wrinkle and whiten in a long hot bath. Thus, the term lichenification is used to describe the pale, orangepeel appearing, thick skin of the vulva that is seen in LSC. Other associated findings are decreased sensation on the affected skin and a change in skin pigmentation (hypopigmentation or hyperpigmentation).
Summary
Chronic diffuse itching is rarely caused by infectious conditions12 (eg, yeast infection) or premalignant processes such as VIN, which tend to cause focal, unilateral symptoms (Chapter 2). Many overlapping dermatoses are often present in a given patient. The key in deciphering the cause of a patientās
Table 1.2 General vulvar care
| Minimizing Daytime Friction |
| ⢠Liberal use of oil-based creams such as Geneās Vitamin E Cream.a For patients with excessivesensitivity, use Crisco shortening or shay butter.b Reapply throughout the day. |
| ⢠Use cold water after using the bathroom to rinse the area. Ask patients to carry a water bottle for this purpose. Cold water (unlike warm water) stops itching. |
| ⢠Instruct patients to not wipe but pad dry their perineum after washing with cold water. |
| Aborting Bouts of Intense Itching Sensation |
| ⢠Apply deep pressure when faced with an itching sensation rather than rubbing of any kind. |
| ⢠Reapply copious amounts of creams (as above). |
| ⢠Place a bag of frozen peas wrapped in a thin towel over the labial folds and perineum. |
| Aborting Nighttime Scratching During Sleep |
| ⢠Use a sedating agent (titrate slowly to maximal tolerance): |
| Doxepin 10ā50 mg 1ā2 h before bedtime |
| Diphendryamine 25ā50 mg 30 min before bedtime |
| Hydroxyzine 10ā50 mg 2 h before bedtime |
| ⢠Keep nails short and wear white cotton gloves at night. |
symptoms is to use a symptoms-based approach in alleviating symptoms, aborting the itch-scratch cycle, and ultimately promoting the skinās health (Table 1.2). Targeted biopsy can then be used to rule out premalignant processes and to guide additional therapy. The case studies at the end of this chapter will provide a guide to differential diagnosis, relief of symptoms, and treatment approach for patients with LS and contact dermatitis.
Lichen planus, plasma cell vulvitis, and desquamative inflammatory vaginitis
Whereas lichen sclerosis, contact dermatitis, and lichen simplex chronicus are characterized by a chief complaint of itching, lichen planus, plasma cell vulvitis, and desquamative inflammatory vaginitis are primarily associated with symptoms of burning, pain with intercourse, and discharge (Table 1.1).
Lichen planus
Classical lichen planus (LP) is characterized by shiny, flat-topped, firm papules (bumps) on the extremities, trunk, and mucosa. The most common form of this mucocutaneous dermatosis that is seen in gynecology is known as vulvo-vaginalgingival syndrome. Vulvo-vaginal-gingival syndrome typically presents as a single or multiple well-demarcated, intensely red lesions with a reticular appearance.* In cases with extensive vaginal involvement, synechiae and varying degrees of vaginal obliteration are common. While oral lesions can vary from painless white lacy streaks to desquamative gingivitis, vulvo-vaginal lesions tend to consistently show lichenoid inflammation.
While lesions of LP on the mucosal surface are tender, lesions on the extremities (eg, wrist or ankles) are typically nontender. LP is a relatively rare condition (1ā2% of the U.S. population); nevertheless the prevalence of L...