Erosions and Ulcers



Erosions and Ulcers


Libby Edwards

Peter J. Lynch



Many clinicians do not distinguish erosions from ulcerations, but to a dermatologist, these represent separate differential diagnostic categories. An erosion is a superficial defect, with only the epithelium lost from the surface. An ulcer is deeper, with the defect extending into or even through the dermis. Erosions generally are red, and there is no discernible depression in the skin to the touch (Fig. 11-1). Ulcers on mucous membranes most often have a white or yellowish fibrin base and have palpable tissue loss (Fig. 11-2). On dry, keratinized skin, the base of extramucosal ulcers are often red, frequently with adherent yellow fibrin remnants, and erosions and ulcers on dry skin can be covered with a crust (scab) of blood and fibrin. Of course, there is overlap; an ulcer begins as an erosion and becomes an erosion as it heals. An erosion can deepen into an ulcer due to infection, irritating medications, or manipulation.


SECTION A: EROSIONS

Many erosions occur when a blister roof sloughs; therefore, all blistering diseases should be considered in the differential diagnosis when erosions are seen (see Chapter 10).

Usually, when an erosion is produced by a blister, the erosion is round or arcuate (Fig. 11-3), and often some intact blisters can be seen. When produced by trauma, erosions are generally irregular, linear or angular.

Erosive skin diseases can be infectious, immune-mediated, or malignant, or traumatic, such as excoriation from scratching.


Erosive Lichen Planus

Lichen planus (LP) exhibits widely differing morphologies, with erosive lichen planus being the most common noninfectious erosive disease of the vulva. Erosive lichen planus is less common on the penis. Red papules (see Chapter 6) are more common than erosions in men, and wet skin often exhibits white skin lesions (see Chapter 8). Some patients exhibit several morphologies, such as white striae surrounding erosions. There are few studies on genital lichen planus, but data on oral lichen planus are more abundant and relative to anogenital lichen planus, so is often referenced in this chapter.


Clinical Presentation

Erosive vulvovaginal lichen planus does not occur in children, is rare in young adults, and, in women, is primarily a postmenopausal disease. Erosive genital LP is by far more common in women, especially in the dermatoses, lichen planus of the penis occurs primarily in uncircumcised males.1 A series of 89 patients with penile lichen planus showed that very few of these were the erosive variant.2 The vast majority of women with erosive vulvovaginal lichen planus have no involvement of extragenital, extramucosal sites. Patients often report genital pruritus, but rubbing and scratching are often painful, and burning, irritation, rawness, dysuria, dyspareunia, and postcoital bleeding are predominant symptoms. Both men and women with erosive genital LP often report oral symptoms of rawness and pain, especially with hard food such as chips, and spicy and acidic foods. Two-thirds to three-quarters
of women with erosive vulvar lichen planus have oral involvement, but the frequency in men is less well documented.3 A series of men with penile LP, mostly nonerosive, had no extragenital involvement including no oral disease in 88.8%.2











The classic lesions of lichen planus on extramucosal skin are pruritic, violaceous, flat-topped papules (see Chapter 5), but these are rare in patients with erosive genital LP. The most common morphologies of mucous membrane LP are white lacy or fernlike striae, especially in the mouth, or erosions, particularly on the non-hairbearing skin of the vulva and the glans and the ventral prepuce of the uncircumcised penis. White morphology is discussed in Chapter 8.











Erosive LP typically affects the modified mucous membranes of the vulva is usual, with bright red, thin epithelium and erosions often most marked in the true mucous membranes of the vestibule, but frequently include the labia minora (Figs. 11-4, 11-5, 11-6).
Normally, these are nonspecific and indistinguishable from pemphigus vulgaris, mucous membrane pemphigus, or an acute irritant contact dermatitis. Sometimes, the erosions are surrounded by white epithelium that can represent the white morphologic variant of LP (Figs. 11-7 and 11-8). However, any mucous membrane erosion is likely to produce a white border due to nonspecific inflammation of adjacent denuded skin. However, when nearby reticulate white lacy plaques are present, the diagnosis is clearly that of lichen planus (Figs. 11-9 and 11-10). In 1982, Pelisse and associates described the vulvovaginal-gingival syndrome, introducing erosive lichen planus as a common subgroup in women.4 Char-acteristically,
these patients suffer erosions of the vulva, vagina, and oral mucosa, especially the gingivae, which otherwise is not a common location for LP (Figs. 11-11 and 11-12). The peno-gingival syndrome is less common but recognized as well (Figs. 11-13 and 11-14).5































Erosive lichen planus often produces remarkable scarring, even when activity of the disease seems low (Fig. 11-15). Labial resorption and scarring of the clitoral hood over the clitoris are common. Narrowing of the introitus is also a frequent complication that can result eventually in urinary retention.



























Vaginal involvement is very common in women with erosive vulvar lichen planus. This is discussed further in Chapter 14, Vaginitis and Balanitis. This ranges from discrete erosions to generalized erythema, both accompanied by an inflammatory vaginitis (Figs. 11-11, 11-16, and 11-17). Rarely, vaginal lichen planus occurs in the absence of vulvar involvement, when the diagnosis is much more easily missed. Erosive vaginal lichen planus produces purulent vaginal secretions that incite an irritant contact dermatitis in the vestibule and worsen the symptoms of vestibular lichen planus. Vaginal adhesions occur with severe disease, sometimes producing obliteration of the vaginal space that makes sexual intercourse or the introduction of a speculum impossible.











Penile lichen planus is less often erosive, but when erosions occur, they can be very painful (Figs. 11-13, 11-18, and 11-19). These erosions may be associated with oral lesions and run
a similar prolonged clinical course to that seen in women.2 Men with erosive penile lichen planus also experience scarring, with phimosis if uncircumcised, or obliteration of the sharp distinction of the glans from the shaft in the circumcised penis. Perianal lesions sometimes occur in both genders, but erosive disease of this area can be subtle and the area should be specifically examined (Fig. 11-20).











Gingival lesions can be localized or generalized, primarily manifested by tender erosions and desquamative gingivitis, sometimes with surrounding solid or reticulate white papules (Figs. 11-11 and 11-13). Classic and more common are linear reticulate papules or erosions on the buccal mucosa, and the tongue is often affected as well (Figs. 11-21 and 11-22). Esophageal lichen planus is increasingly recognized and is likely more common than the literature suggests. Strictures producing dysphagia and weight loss are suggestive of this involvement, and squamous cell carcinoma (SCC) of the esophagus is a rare but serious complication.6 Esophageal scarring predisposes to reflux disease, and reflux worsens esophageal lichen planus, confounding the diagnosis and biopsies at times. Conjunctival
involvement and lacrimal duct scarring can occur.7 Much less common is involvement of the extra-auditory canals, primarily manifested by otorrhea and hearing loss, and rarely resulting in obliteration of the canal.8
















Squamous cell carcinoma (SCC) sometimes occurs in oral, vulvar, and penile lichen planus. Recent information suggests that vulvar SCC is less common that previously believed.9 A survey of 13 100 women with all forms of lichen planus showed twice as many vulvar and esophageal malignancies as expected, but no increase in the risk of vaginal cancer.10 Cancer of the lip was increased 5-fold, the tongue 12-fold, and the oral cavity 8-fold.10 Although probably less common than in lichen sclerosus, SCC is an event we also have seen in women with severe, long-standing lichen planus, so clinicians should be aware. Malignancy in the setting of penile lichen planus certainly occurs and is reported only in case reports and very small series.

There have been reports for many years of lichen planus occurring more often in patients with hepatitis C as well as and reports discounting this association. A recent survey of over 2000 patients with lichen planus of all types showed the incidence of hepatitis C to be 1.8%, significantly increased over 0.6% incidence in controls, but still uncommonly present.11 The incidences of hepatitis B and cirrhosis were twice that of controls.11 More recently, a study has noted an association of oral lichen planus and hypothyroidism.12 The metabolic syndrome appears to be more common with lichen planus in general, but there are no reports of this association specifically with erosive mucosal LP.13 There also appears to be an association of lichen planus with lichen sclerosus, named an LP/LS overlap. This refers to both diseases occurring in the same patient, most often oral lichen planus and vulvar lichen sclerosus, but sometimes one biopsy-proven genital disease evolving into the other.14 This is a combination that I see frequently.

Finally, unreported but generally recognized by vulvologists is the development of residual pain, both following the successful management of erosive vulvar lichen planus and on uninvolved areas of the vulva in women with erosive lichen planus. In a chart review in the office of one of us (LE) of 114 women with erosive vulvar or vulvovaginal lichen planus, 44 patients experienced clinical clearing of the vulva and vagina. However, 10 of those women reported persistent pain. There were even more patients with residual LP who reported pain in unaffected areas, but these were not included. (Coombs A, Edwards L, presented at XXI World Congress ISSVD, Paris, 2011.) So, in this small review, at least 23% of women with successfully managed LP met the criteria for vulvodynia. I am unaware of this finding being discussed in relation to men with erosive LP, and my numbers of men with erosive LP are small.



Diagnosis

Erosions with accompanying white striae are diagnostic of lichen planus; however, this finding is usually absent on the genitalia. When white striae are seen in the mouth of patients with chronic nonspecific genital erosions, this usually indicates that the genital disease is lichen planus as well. However, the clinician should take care because of the occurrence of LP/LS overlap as noted above.

For practical purposes, chronic, nonspecific vulvar erosions without signs of blisters are nearly always lichen planus. And, when accompanied by oral disease, this is especially true. Similarly, chronic erosions of the glans penis without signs of blistering disease are likely erosive lichen planus. Although a biopsy is useful in ruling out other diseases, many genital specialists report that, on mucous membranes or modified mucous membranes, they rarely have a biopsy of erosive lichen planus returned as diagnostic. The histology generally is interpreted as “lichenoid dermatitis” consistent with but not diagnostic of lichen planus. Lichen planus and a lichenoid medication reaction can produce identical clinical and histologic picture, and even contact dermatitis and some phases of lichen sclerosus and immunobullous diseases can show similar nonspecific findings. The biopsy should be taken from the edge rather than the center of an erosion, and it should be submitted for routine histology. If the biopsy is nonspecific or is suggestive of an autoimmune blistering disease, an additional biopsy of nearby noneroded skin can be submitted in transport media rather than formalin for direct immunofluorescence.

The classic biopsy of erosive lichen planus shows a dense, lymphocytic, dermal bandlike infiltrate extending to and damaging the basal layer (Fig. 11-23). A prominent granular cell layer, hyperkeratosis, and acanthosis are common, but erosive mucous membrane lichen planus often exhibits epithelial thinning and flattening. A biopsy that does not include epithelium cannot provide a diagnosis
of LP. Colloid and Civatte bodies are frequently seen in the lower epidermis and upper dermis, and a direct immunofluorescent biopsy makes these more easily visualized.






Most chronic erosive/blistering skin diseases are in the differential diagnosis of erosive genital lichen planus. Pemphigus vulgaris and benign mucous membrane pemphigoid can be nearly indistinguishable, but these are far, far less common and usually more easily diagnosed by routine and direct immunofluorescent biopsy. Sometimes, white epithelium can resemble lichen sclerosus, but the texture of this white epithelium does not have the characteristic crinkled texture of LS. Toxic epidermal necrolysis, severe erosive candidiasis, and an irritant contact dermatitis to an agent such as podophyllum resin can mimic erosive lichen planus, but these are acute rather than chronic in onset. Like erosive lichen planus, plasma cell mucositis also exhibits red, moist, glistening papules, and plaques that may appear erosive. The histology usually white, lacy striae of genital skin are pathognomonic for genital lichen planus. Unfortunately, this differentiates lichen planus from plasma cell vulvitis or balanitis, but many feel that LP and plasma cell mucositis are at least related if not the same disease. Chronic graft vs host disease is indistinguishable clinically and histologically from lichen planus, but the history is clearly the differentiating feature.



EROSIVE LICHEN PLANUS Diagnosis



  • Nonspecific chronic erosions of mucous membranes or modified mucous membranes statistically indicate the diagnosis


  • White lacy striae of the mouth strongly suggest the diagnosis by association


  • Pathognomonic lacy, white surrounding striae on anogenital skin confirm diagnosis


  • A biopsy of the edge of an erosion reporting lichenoid mucositis or dermatitis gives presumptive diagnosis in the appropriate clinical setting



Pathophysiology

Although the etiology of lichen planus is not well understood, autoimmune T-cell-mediated destruction of basal cell keratinocytes is believed to be a major factor. Rarely, lichen planus or a nearly identical lichenoid medication reaction is produced by medications, most often from gold, antimalarial drugs, β-blockers and angiotensin-converting enzyme inhibitors. Recent observations included associations with novel drugs such as biologics (eg, tumor necrosis factor antagonists) and immune checkpoint inhibitors.15


Management

Erosive genital lichen planus usually responds fairly well to superpotent topical corticosteroids, local care, and, in postmenopausal women, estrogen replacement (Figs. 11-24 and 11-25). Occasionally, genital erosive lichen planus can be severe and recalcitrant. Erosive oral disease is often more challenging, as are the less common esophageal and rare eye and ear disease. Where topical corticosteroids cannot be applied or where they do not adhere well, such as in the mouth, intralesional or systemic therapy with antimetabolites or immunosuppressive drugs is sometimes used for persistent disease.












Data regarding the benefit of therapy are poor. A Cochrane Database review showed no convincing evidence for benefit of any therapies16; however, clinicians see very significant improvement in patients with some therapies, especially corticosteroids. Although medications do not cure the disease, lichen planus in most patients is managed quite well with topical corticosteroid therapy. Circumcision is sometimes curative in men.



EROSIVE LICHEN PLANUS Management

Topical therapy



  • Superpotent corticosteroid ointment twice daily


  • Calcineurin inhibitors (tacrolimus or pimecrolimus) twice daily


  • Local supportive care—dilators, infection control, avoidance of irritants


  • For postmenopausal women with vaginal lichen planus—estrogen replacement

Intralesional corticosteroids



  • Triamcinolone acetonide 10 mg/cc into recalcitrant lesions

Systemic therapy occasionally (poor data)—added to topical, intralesional therapy



  • Hydroxychloroquine 200 mg orally twice daily


  • Methotrexate up to 25 mg orally weekly


  • Mycophenolate mofetil up to 1500 mg twice daily


  • Many other immunosuppressives and antimetabolites from case reports


Nonspecific Therapy

Avoidance of irritants such as soap and overwashing, panty liners, and unnecessary topical medications is useful. Topical emollients such as petrolatum are soothing, as are measures such as air-drying rather than towel-drying. Because vulvovaginal lichen planus occurs primarily in the postmenopausal age group, topical or systemic estrogen replacement can be crucial, avoiding an additional and easily corrected cause for mucosal thinning. Estrogen replacement is discussed in Chapter 14, Vaginitis and Balanitis. Women with vaginal lichen planus or with introital narrowing should be counseled in the use of vaginal dilators to use several times a week to prevent continued tightening of the vaginal canal, and men should be encouraged to retract the prepuce daily to minimize the risk of phimosis.


Topical Therapy

The overwhelming first-line therapy for genital lichen planus is topical corticosteroid therapy. Superpotent topical corticosteroid ointments (eg, clobetasol propionate 0.05%, halobetasol propionate 0.05%) applied to vulvar or penile lesions twice daily and covered with a small amount of petroleum jelly can be beneficial. Because occlusion enhances the potency of medication, recalcitrant lichen planus of the circumcised glans can be treated carefully with corticosteroids under condom occlusion for short periods with careful follow-up. Ointments are by far the vehicle of choice; creams, gels, lotions, and solutions are to be avoided because of the irritation induced by the additives regularly contained in these vehicles.

There are no corticosteroids formulated for the vagina. However, small amounts of a corticosteroid ointment can be inserted with a vaginal applicator intravaginally overnight. Alternatively, a commercially available 25-mg hydrocortisone acetate rectal suppository can be inserted vaginally, or hydrocortisone acetate foam designed for hemorrhoids can be inserted into the vagina with a finger. However, these preparations are very low potency. More potent vaginal suppositories can be compounded, with hydrocortisone acetate 100-300 mg suppositories sometimes used, or with insertion of superpotent corticosteroids into the vagina. However, significant absorption can occur, and there are no safety data on vaginal administration. I obtain cortisol stimulation on women using intravaginal superpotent corticosteroids more often than thrice weekly, because I have seen clinically cushingoid features occur in women using daily superpotent vaginal corticosteroids. Also, intravaginal corticosteroids sometimes precipitate vaginal candidiasis, so weekly fluconazole, twice or thrice weekly insertion of a topical antifungal cream, or a warning to the patient to call if sudden itching occurs is important.

The mouth should be addressed as well. I often find oral lichen planus to be a greater challenge than genital lichen planus, and dentists in general do not aggressively treat this miserable condition. There are several suboptimal options for the delivery of a corticosteroid to the mouth. Dexamethasone elixir can be used in a swish, hold as long as possible, and spit fashion. Because topical corticosteroids in the mouth increase the occurrence of Candidiasis, mixing the dexamethasone half and half with nystatin oral solution decreases that risk but also decreases the potency of the corticosteroid. The patient should not eat or drink for 30 minutes after use. Alternatively, clobetasol gel 0.05% can be applied to affected areas of the mouth. Covering the gel with a wet paper towel can hold the medication again the mucosa for longer and increase the effect. Both of these methods of use are started at about four times a day, and then decreased when the disease is better controlled to the least frequent use that maintains comfort.

Some have advocated the use of dental trays to hold medication against the affected gingivae. Dentists who make mouth guards from individualized molds for patients can do this and then leave intact rather than trim the edge that covers the gingivae from the guard. When the disease is better controlled, the frequency of
application is decreased to the least frequent use that maintains comfort.

Second-line therapy for erosive mucosal lichen planus consists of the topical calcineurin inhibitors pimecrolimus 1% cream and, especially, tacrolimus 0.1% ointment.17 Tacrolimus suppositories for the vagina can be compounded as well, and compounding pharmacies have these recipes. However, these medications are not approved by the U.S. Food and Drug Administration (FDA) for lichen planus, and although generally well tolerated in the mouth, usually produce unacceptable and long-lasting burning when applied to inflamed genital skin. In addition, improvement is delayed when compared to topical corticosteroids, and they are blackboxed by the FDA for a risk of producing skin cancer and lymphoma, worrisome to patients who are already at risk for transformation to SCC. The occurrence of these malignancies does not seem to have actually appeared in response to use of topical calcineurin inhibitors. However, these authors and many of our colleagues have not found these medications to be particularly useful for genital erosive lichen planus.


Intralesional Therapy

Corticosteroids injected into recalcitrant mucosal lesions often prompt excellent healing when topical corticosteroids fail (see Chapter 4). Triamcinolone acetonide 10 mg/cc is the usual preparation, using about 0.1 cc/cm2. The oral epithelium can be anesthetized with an oral anesthetic compounded by dentists to produce prompt and complete anesthesia, resulting in a painless injection. However, this topical anesthetic should be avoided on genital skin, where it sometimes produces sloughing. Compounded tetracaine 6%/lidocaine 6% applied for 15-30 minutes is far better tolerated and affords fairly good anesthesia on genital skin.

Intralesional corticosteroids can be used by gastroenterologists during endoscopy, and often in combination with dilation, for chronic recalcitrant esophageal disease as well.18


Systemic Therapy

The only predictably beneficial systemic therapy for erosive lichen planus is systemic corticosteroid therapy, especially prednisone or prednisolone. Dosing of prednisone 40-60 mg/d effects significant and rapid improvement in most patients, but the condition recurs when the medication is stopped. Clearly, the toxicity of chronic systemic corticosteroid therapy prevents this treatment for anything except brief use for flares of disease. Prednisone can be used initially in patients with severe erosions to clear skin sufficiently before transitioning to topical therapy. Alternatively, corticosteroids can be administered by intramuscular injection with triamcinolone acetonide, 40-80 mg. One regimen consists of a monthly injection for 3 months in an attempt to interrupt the disease process and trigger long-lasting benefit (personal communication, Lynnette Margesson, MD).

However, other immunosuppressive medications that are safer for chronic use sometimes appear to produce modest improvement in lichen planus, although the data from controlled trials are lacking. The onset of benefit of these treatments is slow, requiring at least 3 months to produce full effect, and these therapies improve lichen planus less predictably. Hydroxychloroquine 200 mg twice a day is a time-honored inexpensive therapy with minimal laboratory monitoring, and a recent letter reported that 60% of 15 patients experienced an undefined good response.19 Methotrexate 25 mg weekly is another cost-effective therapy, but again with very little data. A letter describing a comparison between methotrexate and mycophenolate mofetil in the treatment of vulvar lichen planus in 44 patients showed comparable benefit using doses of methotrexate mostly <25 mg/wk (70% responded) and mycophenolate mofetil up to 2500 mg/d (64% responded). The degree of response was not reported.3 There are allusions to benefit from oral retinoids such as isotretinoin 40-80 mg/d and acitretin at about 25 mg/d including a recent case report of benefit in penile LP,20 but side effects including teratogenicity require careful monitoring. Other systemic treatments used historically include oral griseofulvin, azathioprine, cyclophosphamide, azathioprine, dapsone, and metronidazole with anecdotal benefit in some patients; clinician familiarity with these medications for appropriate monitoring is important. Thalidomide was shown in a small open trial to be as effective as topical steroids for oral lichen planus, but this has not been repeated.21 There are several case reports of oral cyclosporine being used for lichen planus, and topical cyclosporine swish and spit for oral LP. An unusually well-designed 2013 open label trial examined the effect of apremilast, an oral medication for psoriasis, on LP. Ten patients with moderate to severe LP (only one with mucosal disease) showed significant improvement in 30% of patients.22 However, there have been only six positive case reports since that time and no studies. The JAK inhibitor tofacitinib has been reported in several case reports of lichen planopilaris (LP of hair follicles) and LP of nails, and I have treated four patients with recalcitrant and life-ruining disease with tofacitinib 5 mg twice daily; three cleared by at least 95%, and one patient experienced no improvement. Obtaining this prohibitively expensive medication for LP when not approved for use for any dermatologic indications has proved to be nearly impossible. Potential side effects include intestinal perforation, immunosuppression, and thrombosis. However, trials on other skin diseases are showing good effects, and other systemic and topical JAK inhibitors are available and in development. A large randomized study on the systemic JAK inhibitor ruxolitinib for graft vs host disease, a condition with clinically
and histologically identical skin findings, showed robust data showing benefit.23 And, the JAK inhibitor ruxolitinib has just been released as a cream for atopic dermatitis. The current price is about $2000 for a large tube. There have been reports of some of the biologic agents, primarily adalimumab and ustekinumab, providing some relief for patients with lichen planus, but there are also reports of these medications producing paradoxical lichenoid skin reactions. Other than systemic corticosteroids, there are no systemic therapies known at this time to produce consistent good improvement in resistant erosive genital lichen planus, but trial and error treatment often yields therapies that improve disease when added to good topical and intralesional corticosteroid therapy and local care.


Surgery

Circumcision is the only surgical procedure that sometimes improves the mucosal lichen planus. However, surgery is occasionally needed to separate vaginal adhesions, reverse tightening of the introitus, or to uncover a buried clitoris. Narrowing of the vaginal introitus occassionally progresses to complete closure, resulting in urinary retention requiring urgent surgery. Skin disease should be controlled as well as possible before surgery is contemplated, and great care has to be taken postoperatively to prevent rapid scar reformation. Use of topical steroids and dilators is advisable.

Erosive anogenital lichen planus is chronic and painful, and there are no standard treatment measures that help all patients. Nor are there any high level controlled trials that evaluate therapy for anogenital lichen planus. However, experience suggests that there are various therapies that help some patients. Occasionally, erosive anogenital lichen planus remits, but this is the exception. Also, because of the risk of SCC, patients should be checked regularly throughout the course of the disease. Indurated erosions and ulcers should be biopsied, as should chronic hyperkeratotic areas.

In summary, most patients do well simply with superpotent topical corticosteroid ointments applied twice daily, covered with petroleum jelly, and tapering the frequency of application when skin disease improves. Adjunctive therapies are added to corticosteroids rather than replacing them. Local care is important, such as insertion of a dilator in women with vaginal involvement or introital narrowing to minimize scarring, and careful daily retraction of the prepuce in men to prevent phimosis. Estrogen replacement in postmenopausal women is imperative, and infection control in both sexes is key as well. Those with prominent pruritus should receive bedtime sedation to prevent nighttime scratching. Careful follow-up for early signs of malignant change and side effects of medication is crucial.

Vulvologists have noted residual pain in many women successfully treated for erosive vulvovaginal lichen planus, showing that lichen planus can trigger vulvodynia. Therefore, those patients who continue to report burning and rawness should be re-evaluated for active lichen planus rather than simply treated for this LP more aggressively. Often, medication for neuropathic pain and physical therapy directed at management of vulvodynia precipitated by lichen planus should be the next step, while continuing to medications that control the underlying erosive LP.


Plasma Cell Balanitis and Vulvitis (Zoon Balanitis/Vulvitis)

Zoon mucositis is discussed primarily in Chapter 5. Zoon balanitis or vulvitis (also called balanitis or vulvitis circumscripta plasmacellularis, inflammatory erythroplasia) is a characteristic nonblanching deep red, rusty/orange, or brown red patch on a mucous membrane that shows increased plasma cells on biopsy. The cause is unknown, but the similarity in appearance and histology causes some to suspect a relationship to lichen planus.

Plasma cell vulvitis and balanitis present as a usually solitary plaque on the glans penis or vulva. Although this often appears erosive, the epithelium generally is atrophic but intact (Figs. 11-26 and 11-27). Zoon is most often uncomfortable, with patients describing burning or irritation, and less often itching or no discomfort.

In men, circumcision generally is curative. Otherwise, potent topical corticosteroids are standard first-line therapy that offers some symptomatic relief and improvement in appearance in many patients. As for other dermatoses, relapse is the rule on discontinuation of treatment. Topical tacrolimus, pimecrolimus, and masoprocol have been useful in some, but reported results of therapy are conflicting and I find tacrolimus/pimecrolimus to be
poorly tolerated due to local burning with application. Topical corticosteroids mixed with fusidic acid, imiquimod, intralesional corticosteroids, and CO2 laser have been reported beneficial at times. Although a differentiating feature from lichen planus is sometimes said to be lack of scarring, I have had patients with Zoon vulvitis experience loss of labia minora and clitora phimosis. Also, there are several reports of SCC in situ (intraepithelial neoplasia) in a setting of plasma cell balanitis, so that ongoing surveillance is recommended.












Genital Skinfold Fissures

Genital skinfold fissures are a physical finding rather than a disease; these are a common nonspecific feature of several inflammatory genital diseases, seen primarily on the vulva and perianal skin. Patients sometimes describe these fissures as “paper cuts,” and the clinician has the task of finding the cause and management.


Clinical Presentation

These linear erosions occur within skinfolds, especially the interlabial folds and edges of the clitoral hood in women, in the coronal sulcus and skin wrinkling of the penile shaft, and within the normal skin markings of the perineal body and perianal skin of both men and women (Figs. 11-28, 11-29, 11-30, 11-31, 11-32). They often are transient so that the diagnosis is made by the history in association with thin, red lines in the areas of fissuring, or by having the patient return as soon as fissures recur. Sometimes, magnification is required for identification. At other times, the fissures are so deep as to actually represent ulcers.

Patients report irritation as well as stinging that is accentuated when urine, semen, medicated creams, or sometimes even water touches the area.








Diagnosis

The recognition of skinfold fissures is made by visualization of the finding. The diagnosis of the underlying cause is sometimes obvious, but sometimes difficult and occasionally no cause is found. When acute, Candidiasis is the most common underlying diagnosis. Herpes simplex virus is an uncommon cause of an acute fissure in men and women and, rarely, a bacterial infection, either Staphylococcus aureus or, less often, Streptococcus spp. cause fissures. Although infection with group B Streptococcus has been reported, this is usually Streptococcus pyogenes, and this is often in association with inflammatory vaginitis.
When chronic, noninfectious inflammatory dermatoses are most likely, especially lichen simplex chronicus and lichen sclerosus, although contact dermatitis, psoriasis and any other cause of inflammation can cause fissures less often. Intraepithelial neoplasia and high-grade squamous intraepithelial lesions have been associated with fissure. Maddeningly, at times there is no accompanying skin disease, and cultures, molecular studies and cultures are negative.













Pathophysiology

Presumably, delicate genital skin becomes even more friable when the skin becomes inflamed, and rather than bending in folds, the epithelium cracks. Chronic inflammation often prompts skin to thicken so that it bends less easily, and breaks. Also, many people experience itching with inflammation and resulting rubbing can produce fissures.












Management

For skinfold fissures, the identification, treatment, and then suppression of the underlying condition usually eliminate the fissures. When no underlying factors are identified, an empiric trial of a topical corticosteroid ointment such as clobetasol or halobetasol, covering with fluconazole 150-200 mg every 4-7 days for several weeks, manages most causes of fissures. After the skin has healed, the medications can be gradually discontinued, although recurrence is common.


Mechanical Vulvar Fissures

Mechanical fissures consist of splits occurring at the introitus, usually at the posterior fourchette and primarily with coitus.


Clinical Presentation

Mechanical fissures occur most often in premenopausal women and almost exclusively in sexually active individuals. The onset is usually sudden, without preceding trauma or infection; stinging and splitting of the posterior fourchette occurs during sexual intercourse. Semen, water, and urine burn on contact. The area usually heals quickly but recurs with most acts of intercourse.

On clinical examination, there is a linear erosion at the midline posterior fourchette that may be thin and subtle, or relatively wide, obvious, and nearly ulcerative
(Figs. 11-33, 11-34, 11-35). Some patients exhibit fairly longlasting erythema of the area after healing. Posterior fourchette fissuring is easily seen when the patient presents within a day or two following sexual intercourse. Rarely, women experience erosions or ulcerations that are not at the 6 o’clock position, and in my experience, these are usually at 3 or 9 o’clock. These may be linear fissures, or a small, recurrent, punctate ulcer (Fig. 11-36).
















Some women report associated pain distinct from the fissure but within the vestibule. Some clinicians postulate that fissuring occurs in association with vulvodynia or vestibulodynia (previously called the vulvar vestibulitis syndrome) (Chapter 13).



Diagnosis

The diagnosis of a recurrent posterior fissure is easily made by the morphology and history. However, the clinician should take care to visualize the fissure either in the office or by photograph. Some patients with vulvodynia/vestibulodynia describe fissuring with intercourse, but when seen following sexual activity in the office, the patient and provider learn that although the pain feels like splitting, there is no actual tearing. The diagnosis is not that of a mechanical fissure, but rather vestibulodynia. Additional diseases to consider include HSV infection because of the same-site erosions. However, this should
not occur with each act of coitus, and molecular studies for HSV will be negative.








MECHANICAL VULVAR FISSURES Diagnosis



  • Both history and visualization of a same-site fissure immediately following sexual activity



Pathophysiology

The cause of mechanical fissures is not known. Although some clinicians have implicated low estrogen levels as a cause of posterior fourchette fissuring, there are no clinical signs of this, and the addition of oral or topical estrogen replacement is not helpful. Some believe that pelvic floor abnormalities may contribute to both vestibulodynia and posterior fourchette fissuring, helping to explain a possible association of these two conditions. Lichen sclerosus predisposes to posterior fourchette fissuring, and this fissuring resolves with treatment of the lichen sclerosus. Most women simply and relatively suddenly experience the onset of posterior fourchette fissuring with no known cause or accompanying abnormalities. Interestingly, a condition termed a perineal groove is seen rarely in newborn girls, and it generally heals in the first year or two.24 This is described as a wet, red groove from the posterior fourchette to the anus at 12 o’clock, and this can be the entire length or partial length. This is postulated to be a self-healing congenital malformation; perhaps there is a relationship.


Management

Liberal lubrication during sexual activity, topical anesthetics such as lidocaine jelly 2%, and woman on-top positioning minimizes fissuring for some women, but this does eliminate the problem. Avoiding sexual activity to allow the area to heal well is not useful. Primary excision with suture closure generally worsens the fissuring by narrowing the vaginal opening.

One management option consists of the patient applying a topical anesthetic such as lidocaine or, more effective, compounded tetracaine 6%/lidocaine 6%, then produce the fissure with sexual activity. Then, keep the fissure open with the frequent use of dilators, so that reepithelialization occurs across the surface of the erosion (Hope Haefner, MD, personal communication).

Otherwise, definitive management consists of a perineoplasty. The skin surrounding the posterior fourchette is surgically excised, and vaginal epithelium is advanced to cover the defect. The success of the procedure depends largely on patient selection. Women should be evaluated for the likely presence of vestibulodynia before surgery, since their pain does not totally remit unless the entire vestibule is removed, or unless they receive medical treatment and/or physical therapy for vestibulodynia in addition to surgery.



MECHANICAL VULVAR FISSURES Management



  • Careful visual evaluation for underlying skin disease such as lichen sclerosus


  • Replace estrogen if atrophic wet mount


  • Lubrication before sexual activity


  • Patient produces the fissure, then keep it open with careful perineal massage with petroleum jelly and dilators, to allow the fissure to re-epithelialize and heal in the open position


  • If unsuccessful, perineoplasty; avoid simple excision with suture closure


Erosive Lichen Sclerosus (Lichen Sclerosus et Atrophicus, Hypoplastic Dystrophy)

Lichen sclerosus is a chronic fragile dermatosis characterized by atrophic white plaques, occurring most commonly on the genital skin of both sexes, although there is a female preponderance of 10:1 (see Chapter 8 for primary discussion). Although lichen sclerosus is not primarily an erosive disease, intense itching with resulting rubbing of this fragile skin often produces erosions and purpura. There is fissuring of the anterior midline, as well as vestibular erosions following sexual activity.

The usual presenting symptom of lichen sclerosus is severe pruritus, often leading to painful erosions as this fragile skin is scratched (Fig. 11-37). These excoriations and other erosions resulting from fragility of the skin can be very painful and can predispose to secondary infection.
The atrophic skin fissures more easily, so patients may present with pain on intercourse, and young girls often experience constipation resulting from pain of fissures with defecation.






Eroded lichen sclerosus is sometimes indistinguishable from erosive lichen planus, benign mucous membrane pemphigoid and pemphigus vulgaris, so biopsy and examination of other mucous membranes and skin surfaces may be necessary to make the diagnosis of lichen sclerosus.

Eroded lichen sclerosus is more likely than noneroded disease to develop bacterial or candidal superinfection when a superpotent topical corticosteroid is added for treatment. Early detection and treatment is appreciated, and I sometimes prophylax these women with cephalexin and weekly fluconazole the first week until healing begins.


Lichen Simplex Chronicus (Eczema, Atopic Dermatitis, Neurodermatitis)

This excruciatingly pruritic condition exhibits erosions only secondarily, as a result of rubbing and scratching (see Chapter 5 for primary discussion). Erosions from scratching (excoriations) and fissures occur in many patients (Fig. 11-38). The diagnosis is generally recognized by the history of scratching and the observation of irregular or linear erosions consistent with excoriations on the vulva or scrotum. Therapy includes topical corticosteroids, infection control, and nighttime sedation to minimize scratching during sleep.












Contact Dermatitis

Contact dermatitis sometimes presents with erosion on genital skin; this is often preceded by blistering that is evanescent and unnoticed (see Chapter 10). The most common contact dermatitis to produce genital erosions is a strong irritant contact, which is a chemical burn that can either blister or erode. The erosions are in the distribution of contact with the irritant, usually with surrounding erythema (Fig. 11-39). Because the irritant is strong, the original agent and burning with contact most often is remembered.


Fixed Drug Eruption

A fixed drug eruption, discussed primarily in Chapter 10, is a peculiar allergic reaction to a medication that produces same-site blisters that erode into well-demarcated, often round lesions (Fig. 11-40).


Necrolytic Migratory Erythema

This is a very rare condition that is caused by a glucagonoma, an α-cell tumor of the pancreas, which is usually malignant. Serum levels of glucagon are elevated. Patients are usually ill with weight loss, diarrhea, malabsorption, and diabetes. This rash can also be seen with liver disease, inflammatory bowel disease, pancreatitis, malabsorption disorders (ie, celiac sprue), and other malignancies, when the eruption is termed pseudoglucagonoma syndrome.

The rash begins as erythematous papules around orifices and flexures, including those of the genitalia. These
papules coalesce into plaques, and central erosions occur and crust, eventually producing a circinate migratory erythema. Histology of the skin shows superficial necrolysis and infiltration with lymphocytes. Treatment consists of removal of the tumor, if possible, or correction of the underlying cause of the pseudoglucagonoma syndrome. Topical corticosteroids may improve symptoms of the rash, but they do not clear it.







Malignancies Presenting as Erosions


Basal Cell Carcinoma

Basal cell cancers are associated, classically, with sites of sun exposure, but 10% occur on nonexposed sites and account for 5% of genital cancers (see Chapter 7). The lesions often display the typical pearly, rolled edge, with a central depression or necrotic erosion (Fig. 11-41). The tumors are more fragile than normal, nonneoplastic skin, and the skin erodes as they outgrow their blood supply, especially in this area of friction. They invade locally, but almost never metastasize. The diagnosis is made by biopsy, and management consists of conservative local excision. Those in more difficult sites may be amenable to radiotherapy.







Invasive Squamous Cell Carcinoma

Squamous cell cancers account for more than 90% of genital cancers and often arise in the site of chronic scarring or inflammation such as lichen sclerosus and lichen planus (see Chapter 7 for primary discussion) or as a result of high risk human papillomavirus infection (Fig. 11-42). The lesions present as red or skin-colored plaques or nodules that erode and eventually ulcerate as they enlarge. The diagnosis is made by biopsy, and therapy initially is managed by a surgical oncologist.


Intraepithelial Carcinoma (Differentiated Vulvar/Penile/Scrotal/Anal Intraepithelial Neoplasia, High-Grade Squamous Intraepithelial Lesion, Bowen Disease, Bowenoid Papulosis, Squamous Cell Carcinoma In Situ, Erythroplasia of Queyrat)

Histologic SCC in situ is termed differentiated intraepithelial neoplasia when associated with lichen sclerosus or lichen planus or high-grade squamous intraepithelial lesion when associated with human papilloma virus infection. See Chapters 5 and 7 for primary discussion. These conditions can present with multiple morphologies, including skin-colored, white, or brown papules and
plaques. The appearances illustrated here are erosive and ulcerative (Fig. 11-43).

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Related posts:

  1. Edema
  2. Vaginitis and Balanitis
  3. Special Issues in Genital Dermatology
  4. Red Disorders: Patches and Plaques

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Jan 8, 2023 | Posted by in GENERAL | Comments Off on Erosions and Ulcers

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