MUCOSAL DISEASE: ORAL AND GENITAL

Dec
2015
Vol. 34. No. 4

Introduction

There is a wide range of inflammatory and neoplastic disorders that affect mucosa of the oral cavity. Many of these conditions manifest in the oral cavity and the anogenital region, some affect the oral cavity and skin only, and others are unique to the mouth. Clearly the conditions that occur on the skin and mouth will be familiar to most dermatologists, but others occur specifically on mucosa without cutaneous involvement and may be new and unfamiliar. Even for those conditions that affect the skin and mucosa, the clinical presentation in the mouth frequently differs from their cutaneous manifestation. Awareness of the similarities and differences will help the clinician achieve the diagnosis and select the correct therapy.

White lesions are common in the oral cavity and despite often similar clinical appearances, include a range from variants of normal (leukoedema) to mucocutaneous immunological disorders (oral lichen planus) to premalignancy and cancer. Drs Jones and Jordan discuss many of these conditions focusing on the clinical presentation, methods to establish the diagnosis, and practical solutions to manage the disorders. Ulcers are also common in the oral cavity and like white lesions encompass a range of immunologic and neoplastic disorders. Importantly for the clinician, oral squamous cell carcinoma is common with biopsy invariably required to both establish the diagnosis and exclude other similar-appearing, traumatic, immune-mediated, and fungal disorders. The vesiculobullous disease mucous membrane pemphigoid and pemphigus vulgaris frequently present as ulceration without bullae and these disorders are discussed in this context.

HPV infection produces many benign papillary growths; but increasingly, oncogenic strains, mostly HPV 16, are the cause of a rising incidence of carcinomas in the tongue and oropharynx. Dr Yom discusses the rising incidence of this disorder, how to establish the diagnosis, adjuvant testing, and the role of radiation therapy in its management. The clinical profile of patients with HPV-associated oropharyngeal cancer (OPC) differs quite notably from that of traditional head and neck cancer patients, and the prognosis for HPV-associated OPC is significantly better. We hope that this issue will assist clinicians as they see patients with oral lesions and those with conditions of the anogenital mucosa.

It can be fun, surprising, and discouraging to practice a new and often unrecognized subspecialty. Genital dermatology is actually a multidisciplinary area including: dermatology, gynecology, physiatry, neurology, and psychiatry, in addition to the well-recognized and studied diseases that fall into the fields of infectious disease and oncology. Chronic genital symptoms are usually multifactorial
in nature, with skin diseases complicated by infection, irritant contact dermatitis, estrogen deficiency, and psychological factors. Many practitioners are uncomfortable treating these patients. Skin diseases present atypically in skin-fold areas, pain syndromes are very common, and discomfort of using potent topical corticosteroids and chronic therapy all make a provider unsure and unassertive. Fortunately, careful and caring management of these patients is appreciated enormously and usually very effective, and patients are forgiving of trial and error therapy. Even without a specific proven diagnosis, attention to detail, vigilant follow-up, empiric therapy after careful elimination of infections and malignancy usually provide patients with significant relief.

The articles in this issue target common, unrecognized problems such as the pain syndrome vulvodynia, skin diseases such as lichen planus, and the distinctive but rarely discussed problem of noninfectious ulcers. Hopefully, these discussions will pique the interest of the clinician, who will then teach theirself, with the help of the patients.

The information on vulvodynia is arguably the most useful in this issue for the average provider. Nearly 20% of women experience this pain syndrome at some point in their life, and 7%-8% of women have unexplained sensations of burning and irritation on any given day. Therefore, these women are seen very often, but usually treated repeatedly and ineffectively with antifungal and antibiotic
therapies without culture proof of infection. The dialogue in this issue provides the clinician with effective tools for managing the patient with symptoms but no objective abnormalities.

Much less well-recognized than vulvodynia are penodynia, scrotodynia, and anodynia; but Dr Edwards and other providers have extrapolated information on vulvodynia for successful management of many of these unfortunate men.

Another article in this issue that can be used daily by many providers is an explanation and update on the terminology for genital tumors produced by human papillomavirus (HPV) infections. For years, the terminology was dependent on the specialty. Dermatologists called atypia from HPV infection squamous cell carcinoma in situ, later renamed bowenoid papulosis. Gynecologists talked about vulvar intraepithelial neoplasia (VIN); but this terminology did not differentiate between HPV-related neoplasia and that associated with the chronic underlying diseases of lichen sclerosus and lichen planus, a condition with a much higher risk of invasion and metastasis. Over the past few years, multidisciplinary groups have revised this terminology. The information in this article helps the clinician to understand and interpret the biopsy reports.

The article on erosive lichen planus helps the clinician to recognize the atypical and nonspecific appearance of lichen planus on the genitalia. Importantly, this article contains information regarding the management of other inflammatory chronic skin diseases on the genitalia and in the vagina. Nonspecific local care, estrogen replacement, and the administration of corticosteroids in the vagina
are all discussed and are important aspects of treating many conditions on the genital mucous membranes and modified mucous membranes. These issues are less relevant—and sometimes totally irrelevant—when the diseases occur on extragenital skin.

The risks of severe scarring and secondary squamous cell carcinoma are also discussed.

Finally, the manuscript on noninfectious ulcers addresses a group of diseases that is generally not discussed. The average practitioner begins an evaluation with the erroneous assumption that most genital ulcers are sexually transmitted, either herpes simplex virus (HSV) or syphilis. In reality, HSV in an immunocompetent patient is an erosive, not ulcerative disease. Second, chancres are extremely uncommon in the United States compared to noninfectious causes of genital ulcers. This article gives the reader a manageable differential diagnosis.

In the end, the patient is the best teacher of genital disease for the observant and careful provider. Hopefully, this issue will help to direct and fill in the gaps.

White lesions in the oral cavity: clinical presentation, diagnosis, and treatment

Kyle Burke Jones, DDS | Richard CK Jordan, DDS, PhD, FRCPath

White lesions in the oral cavity are common and have multiple etiologies, some of which are also associated with dermatological disease. While most intraoral white lesions are benign, some are premalignant and/or malignant at the time of clinical presentation, making it extremely important to accurately identify and appropriately manage these lesions. Due to their similar clinical appearances, it may be difficult sometimes to differentiate benign white lesions from their premalignant/malignant counterparts. This review will discuss many of the most common intraoral white lesions including their clinical presentation, how to make an accurate diagnosis, and effective treatment and management strategies.
Semin Cutan Med Surg 34:161-170 © 2015 Frontline Medical Communications

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Differential diagnosis and management of oral ulcers

Amanda Siu, DDS | Daniel M. Ramos, DDS, PhD | Katina Landon, DDS

The diagnosis and treatment of oral lesions is often challenging due to the clinician’s limited exposure to the conditions that may cause the lesions and their similar appearances. While many oral ulcers are the result of chronic trauma, some may indicate an underlying systemic condition such as a gastrointestinal dysfunction, malignancy, immunologic abnormality, or cutaneous disease. Correctly establishing a definitive diagnosis is of major importance to clinicians who manage patients with oral mucosal disease. Some of these diseases are infectious; however, most are chronic, symptomatic, and desquamative. Treatment and management requires an understanding of the immunopathologic nature of the lesion. This review will address how to differentiate and diagnose varying types of oral ulcers and provide a treatment strategy. 
Semin Cutan Med Surg 34:171-177 © 2015 Frontline Medical Communications

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HPV and oropharyngeal cancer: etiology and prognostic importance

Sue S. Yom, MD, PhD

HPV is the most common sexually transmitted disease, but the overwhelming majority of individuals clear the infection. A small percentage of individuals develop persistence of oncogenic HPV types, especially HPV-16; and as a result, squamous cell carcinoma can develop in the tonsils and base of the tongue. Over 70% of oropharyngeal cancers are now thought to be associated with oncogenic HPV infection. Immunohistochemistry for p16 protein is often used as a surrogate marker for oncogenic HPV in the oropharyngeal tissues, although alternative HPV DNA testing methods are under intensive study. The clinical profile of patients with HPV-associated oropharyngeal cancer (OPC) differs quite notably from that of traditional head and neck cancer patients, and the prognosis for HPV-associated OPC is significantly better. As a result, experimental clinical trials are focused on de-intensification of therapies with the hope of preserving an improved long-term quality of life for these patients.
Semin Cutan Med Surg 34:178-181 © 2015 Frontline Medical Communications

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Genital lichen planus: update on diagnosis and treatment

Kathleen Zendell, MD

Lichen planus (LP) is an inflammatory autoimmune disease that affects both glabrous and mucosal skin. Although pathophysiology has not yet been fully defined, LP is a T-cell mediated disorder that demonstrates an increased Th1 cytokine expression as well as T-cell reactivity against basement membrane zone components. In males, genital LP often takes its more classic form as pink, shiny, flat-topped papules on the glans and coronal sulcus. In women, erosive disease is most common and often leads to significant scarring and sexual dysfunction. Therapeutic management is challenging, and control rather than cure is the goal. Topical corticosteroids remain first-line therapy, but some women will require systemic immunosuppressants to achieve remission. Surgery is less common for women with significant scarring who wish to resume sexual activity. Further research is needed on pathogenesis, and randomized controlled trials are necessary to better define best treatments for this chronic disease.
Semin Cutan Med Surg 34:182-186 © 2015 Frontline Medical Communications

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Noninfectious genital ulcers

Carly Kirshen, MD, FRCPC, FAAD | Libby Edwards, MD, FAAD

Noninfectious genital ulcers are much more common than ulcers arising from infections. Still, it is important to take a thorough history of sexual activity and a sexual abuse screen. A physical exam should include skin, oral mucosa, nails, hair, vulva, and vaginal mucosa if needed. The differential diagnosis of noninfectious genital ulcers includes: lipschütz ulcers, complex aphthosis, Behçet’s syndrome, vulvar metastatic Crohn’s disease, hidradenitis suppurativa, pyoderma gangrenosum, pressure ulcers, and malignancies. It is important to come to the correct diagnosis to avoid undue testing, stress, and anxiety in patients experiencing genital ulcerations.
Semin Cutan Med Surg 34:187-191 © 2015 Frontline Medical Communications

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