Hospital Medicine

Diseases of the external genitalia to cervix

I. Problem/Condition.

Diseases of the lower female genital tract may be the primary reason for hospitalization, such as a rectovaginal fistula in Crohn's disease, or a secondary complaint as in vaginal candidiasis in a young woman with poorly controlled diabetes. Some lesions may be found incidentally on an exam done for other reasons.

The differential diagnosis of vulvar, vaginal and cervical disease is quite broad and there are many ways to classify it. Foster, in his review article on the subject suggests dividing the differential diagnosis by location and type of lesion. This can be further stratified by the patient's symptoms and hormonal status. All of the above should help to narrow the differential diagnosis for hospitalists familiar or unfamiliar with gynecologic pathology, and should ultimately guide the appropriate next steps.

A. What is the differential diagnosis for this problem?

VULVAR DISEASE

Note, there is some overlap between vulvar and vaginal lesions. Exceptions are noted.

Macules

  • Acanthosis nigricans: velvety hyperpigmented plaques in skin creases such as the genitocrural folds.

  • Allergic or irritant dermatitis: presents with pruritis or burning pain along with erythema, edema, and inflammation. Of note, may be caused by irritation from urinary incontinence.

  • Atopic dermatitis: pruritis or burning, often found in conjunction with other sites of eczema.

  • Atrophy: pruritis or burning, thinned tissue, may have fissuring.

  • Candida: pruritis or burning, usually in premenopausal females (prefers estrogenized tissue). May also present with erosions or fissures and vaginal discharge Satellite papules are often present.

  • Drug eruption: pruritic or burning erythematous plaques. Has been associated with trimethoprim-sulfamethoxazole, non-steroidal anti-inflammatory drugs (NSAIDs), tetracyclines, metronidazole, and other medications.

  • Lichen simplex chronicus: pruritis and burning. Lesions appear leathery and thickened due to chronic trauma Often bilateral and symmetric. May be associated with chronic or recurrent infection, dermatitis, or other irritation.

  • Lichen sclerosis: pruritis, pain, dysuria. Lesions are thin, coalescing, white plaques, sometimes with submucosal hemorrhage and it usually occurs in postmenopausal (or premenarchal) females. It does not extend into vagina. There is a small risk of malignant transformation.

  • Lichen planus: pruritis and/or pain, often with discharge. May present with whitish plaques, erythematous erosions, or violaceous papules, and usually occurs in post-menopausal women. This can extend into the vagina. Tissue is friable resulting in post-coital bleeding or bleeding on exam.

  • Nevus: asymptomatic, but at risk for malignant transformation.

  • Paget's disease: pruritis, burning, bleeding. May be eczematous in appearance.

  • Psoriasis: erythematous well-demarcated patches, often without characteristic scale. Occurs in approximately 20% of women with psoriasis elsewhere and does not extend from vulva into vagina.

  • Seborrheic dermatitis: oily scale on a pale to erythematous base. Edema may be present.

  • Vitiligo: Patches of depigmented skin, may be associated with vitiligo elsewhere on the body.

Papules, nodules or masses

  • Acrochordon: skin tags which are soft, sessile or pedunculated, and hairless.

  • Condyloma acuminata (genital warts): usually asymptomatic, but may be pruritic or painful. May vary from flat papules to verrucous lesions. Caused by human papillomavirus infection.

  • Endometriosis: painful nodules from less than 1cm to several cm in size. The vulva is a rare site of this disorder.

  • Fibroma: asymptomatic unless it is large. A common vulvar mass, usually on the labia majora and less than 10 cm.

  • Fox-Fordyce disease: pruritic apocrine papules with visible inflammation. Improve with menopause.

  • Granular cell myoblastoma: asymptomatic, rare, neural sheath tumor (schwannoma). May resemble squamous cell carcinoma.

  • Hemangioma: usually asymptomatic unless it is large, in which case it may cause discomfort. Can ulcerate or bleed.

  • Hidradenoma: asymptomatic, but can be pruritic or bleed if necrotic. Found on labia majora or minora, well defined, pink to gray in color and generally less than 2cm. Often has white surface epithelium. Almost always found in white females from 30-70 years old.

  • Keratoacanthoma: firm, round papules originating from pilosebaceous glands which rapidly develop into dome-shaped nodules with a central depression. Lesions may look like squamous cell carcinoma and have malignant potential.

  • Lipoma: asymptomatic. Usually on labia majora, and less than 3cm.

  • Micropapillomatosis: asymptomatic normal variant which manifests as symmetric, homogenous fine papules. Not associated with human papillomavirus (HPV).

  • Molluscum contagiosum: multiple asymptomatic papules less than 5mm with umbilicated centers. Viral in nature and is contagious while lesions are present.

  • Neurofibroma: often asymptomatic fleshy brown to red masses in patients with neurofibromatosis.

  • Syringoma: asymptomatic to mild pruritis. Rare cystic adenoma of eccrine glands. May present with multiple papules.

  • Seborrheic keratosis: well-defined raised lesions with a rough surface, often with similar lesions elsewhere on the body.

  • Urethral caruncle: asymptomatic or dysuria, urinary urgency and frequency or generalized pain. Usually in postmenopausal women.

Ulcers

  • Aphthous ulcers: painful, often with oral ulcers, but does not meet criteria for diagnosis of Behcet's syndrome.

  • Behcet's syndrome: painful and in combination with oral ulcers and other signs to confirm the diagnosis of this vasculitis.

  • Chancroid: from Haemophilus ducreyi. Starts with a painful papule which turns into a painful ulcer over several days; often more than one ulcer. Buboes or tender lymph nodes may be present. Sexually transmitted, but not common in the United States.

  • Cicatricial pemphigoid: pruritis and pain, usually in postmenopausal women, and ulcers can extend into vagina. Also presents with vaginal desquamation and conjunctivitis.

  • Crohn's disease: painful ulcers located in intralabial sulcus, perineum or intracrural folds. May also present with rectovaginal or recto-Bartholin fistulas, and may precede intestinal symptoms.

  • Decubitus ulcer: painful (unless patient has a loss of sensory function). Usually extend into vulva from ischial tuberosities, sacrococcygeal area, or urethra.

  • Epstein-Barr virus: multiple painful ulcers in the setting of acute infectious mononucleosis.

  • Granuloma inguinale (donovanosis): from Klebsiella granulomatis. Painless slowly progressive ulcers without buboes. Ulcers may bleed. Sexually transmitted and very rare in the United States.

  • Herpes simplex virus (HSV): multiple painful ulcers or vesicles.

  • Lymphogranuloma venereum: from Chlamydia trachomatis strains, generally uncommon in the United States. Starts with painless papule which turns into a self-limited painless ulcer. Unilateral buboes may be the presenting complaint. Can also have secondary bacterial infection of ulcers. Sexually transmitted.

  • Pyoderma gangrenosum: painful single or multiple well-defined ulcers, usually in areas of vulva that have had minor injury (pathergy). May present in conjunction with a systemic illness, including inflammatory bowel disease, rheumatologic disease, or malignancy.

  • Stevens-Johnson syndrome or toxic epidermal necrolysis: painful erosions in conjunction with oral ulcers along with other diagnostic criteria for the disorders.

  • Syphilis: painless chancre caused by Treponema pallidum. Sexually transmitted. May also be found in the vagina or cervix.

Cysts

  • Bartholin's duct cyst: often asymptomatic and requires no treatment unless infected. Usually unilateral and fluctuant. Gland is located between vestibule and labia majora. Can have malignancy of Bartholin's duct.

  • Skene's duct cyst: can be asymptomatic or cause discomfort. Duct travels between Skene's gland on the vaginal wall near the urethra and the vulva.

  • Epidermal inclusion cyst: painless unless infected. These common cysts are usually at the site of trauma in the anterior labia majora.

Vesicles or bullae

  • Acrodermatitis enteropathica: erythematous vesiculopapular lesions, or may look more eczematous. In the hospitalized patient this could be the result of nutritional zinc deficiency.

  • Bullous impetigo: vesicles with associated honey-colored scale and crusting. From Staphylococcus aureus infection.

  • Bullous pemphigoid: pruritic lesions usually in prepubertal or postmenopausal females.

  • Bullous systemic lupus erythematosus: pruritic or painful. Relatively uncommon in patients with lupus, but most likely to be found in young African-American women.

  • Paraneoplastic pemphigus: painful and associated with lymphoproliferative tumors including non-Hodgkin's lymphomas and chronic lymphocytic leukemia as well as thymoma and some lung cancers.

  • Pemphigus vulgaris: can be painful. More common in postmenopausal women and lesions can occur on labia minora, vagina and cervix along with generalized symptoms of the disease.

Abscess or cellulitis

  • Bartholin's gland abscess: often unilateral. Gland is located between vestibule and labia majora.

  • Hidradenitis suppurativa: recurrent abscesses of apocrine glands along milk line. There are no apocrine glands on inner labia majora or vestibule.

  • Bacterial abscess, cellulitis or necrotizing fasciitis: painful erythema. Consider necrotizing fasciitis if infection is rapidly spreading or if unstable vital signs. Usually mixed aerobic and anaerobic flora.

Other

  • Edema: can be generalized and spontaneous, from trauma, systemic illness such as renal failure, angioedema, allergy (including to semen), or from infections such as herpes simplex virus, syphilis, or lymphogranuloma venereum.

  • Hematoma: painful. Usually from blunt trauma.

  • Pediculosis pubis: pruritic. Found in areas with hair only, and organism and its eggs can be seen on visual inspection.

  • Pinworm: nocturnal pruritis.

  • Scabies: pruritic, with papules and burrows generalized over body.

  • Vulvar intraepithelial neoplasia: can be light, dark or erythematous in color and raised or ulcerated. Often a delay in diagnosis because of its varied appearance.

VAGINAL DISEASE

Many of the vulvar conditions listed above can be found in the vagina as well. In addition, the following conditions may be found in the vagina.

Masses or cysts

  • Dysontogenetic cyst: usually asymptomatic and may be multiple. Located in the upper half of the vagina.

  • Melanoma: presents with varied appearance, vaginal discharge or bleeding.

  • Inclusion cyst: usually asymptomatic and present from birth or from surgical trauma. This common vaginal cyst is found on the posterior or lateral lower third of the vagina.

  • Squamous cell carcinoma: the most common primary vaginal malignancy. Discharge and/or pain may or may not be present.

  • Urethral diverticulum: presents with chronic or recurrent lower urinary tract symptoms. May not be visible on examination.

Ulcers

  • Foreign bodies: retained foreign bodies such as tampons, diaphragms or pessaries can lead to ulceration from drying and pressure necrosis. May cause malodorous vaginal discharge as well.

  • Atrophy: from estrogen deficiency as above.

Discharge

  • Bacterial vaginosis: usually no pruritis or pain. Thin white discharge uniformly coats vaginal walls and has a fishy odor. Present in sexually active women.

  • Candida: as described above in section on vulvar disease.

  • Cervicitis: see section on the cervix below and see chapter on cervicitis.

  • Desquamative inflammatory vaginitis: purulent discharge, pruritis and/or pain and erythema. Usually in postmenarchal and premenopausal females.

  • Fistula: rectovaginal or vesicovaginal fistulas can lead to chronic vaginal discharge.

  • Physiologic leukorrhea: usually asymptomatic normal whitish discharge in postmenarchal and premenopausal females. May vary in amount and texture throughout the menstrual cycle.

  • Trichomoniasis: asymptomatic or yellow-green malodorous vaginal discharge and vulvar irritation. May see punctate hemorrhages on vagina and cervix. Sexually transmitted.

Other

  • Trauma: lacerations or ulcers from injury or intercourse.

  • Toxic shock: from Staphylococcus or Streptococcus infection.

Masses and cysts

  • Condyloma acuminatum: asymptomatic, often not visible on examination until cervix is swabbed with acetic acid.

  • Endocervical polyp: presents with bleeding, usually after intercourse or on examination. Most common in multiparous women aged 40-50. Usually less than 4cm, smooth, and red to purple in color.

  • Endometriosis: asymptomatic or may cause discharge, dysmenorrhea, and dyspareunia. Red or blue to dark brown on examination, and nonblanching.

  • Myoma: small usually asymptomatic mass which causes symptoms if it is expanding and putting pressure on adjacent structures, causing dysuria, urinary urgency or obstruction, dyspareunia, or cervical obstruction.

  • Nabothian cyst: very common asymptomatic retention cysts, which are translucent or whitish in color and usually 3mm to 3cm in size. Considered normal.

Trauma

  • Laceration: bleeding, usually after childbirth or dilation and curettage.

Cervicitis

  • Infectious cervicitis: from Chlamydia trachomatis, Neisseria gonorrhea, and Mycoplasma genitalium among others.

  • Non-infectious cervicitis: from trauma, foreign objects such as a diaphragm, or from irritants such as douching or latex.

See separate chapter on cervicitis for further details.

B. Describe a diagnostic approach/method to the patient with this problem

The history and physical examination are the most important components of the approach to female patients with problems of the lower genital tract. Both the history and exam should then guide the subsequent workup. Empiric treatment for candida is a tempting solution for hospitalized women with vaginal itching or pain, but given the broad differential diagnosis and the potential for serious illness, it should not be the primary approach to the problem.

1. Historical information important in the diagnosis of this problem.

In taking the patient's history, obtain the following information:

  • Age and hormonal status (premenopausal or postmenopausal)

  • Current symptoms: discharge, pain, burning, itching or systemic symptoms including oral ulcers, rashes, arthritis, gastrointestinal symptoms, etc.

  • Use of any topical products in genital area, such as douches or other irritants

  • Obstetric and gynecological history, including dates of previous deliveries, menstrual history (age of menarche, frequency of menses, any symptoms with menses), any abnormal pap smears, sexually transmitted diseases, or other gynecologic conditions

  • General medical history and, in particular, any history of systemic diseases such as Crohn's, Behcet's or others

  • Current medications, noting if any of them are new medications and including any self-treatment for presenting symptoms

  • Sexual history: current and past sexual activity

  • Safety: any domestic violence, safety in current relationships

2. Physical Examination maneuvers that are likely to be useful in diagnosing the cause of this problem.

In addition to a general examination with particular focus for signs such as oral ulcers, abdominal tenderness, rashes, or other possible systemic components of the diseases listed above, a pelvic exam must be done. The pelvic exam should start with an inspection of the visible external structures, looking specifically for any lesions listed in the section on differential diagnosis above, paying particular attention to their distribution. A speculum should then be inserted to visualize the vagina and cervix. To make the cervix easier to visualize the patient should have an empty bladder.

Some other techniques suggested by Casey et al. include retract the labia majora laterally and encourage the patient to relax the introitus, leave her pelvis and lower back relaxed and on the exam table, place her knees as far out to the sides as possible, and practice deep breathing if the exam is particularly difficult. Try not to force the speculum if the patient is not relaxed. For most women, aim the speculum posteriorly. If the cervix is still difficult to find, it may help to do a bimanual exam to locate it, and then reinsert the speculum.

3. Laboratory, radiographic and other tests that are likely to be useful in diagnosing the cause of this problem.

In general, the following tests may be useful for pathology of the lower female genital tract:

  • Vaginal pH: less than 4.5 is normal; greater than 4.5 can be seen with infections except for candida.

  • Wet prep and microscopy of vaginal secretions.

  • Fungal culture: especially if diagnosis of candida is in question, or if there is concern for Candida glabrata, such as recurrent infection, which may be resistant to typical antifungals.

  • Biopsy: to assist with diagnosis of any atypical or unknown findings, to confirm clinical suspicion of a disease, or to prevent delay in diagnosis of vulvar cancer. This can be done in consultation with the gynecology service.

More specific testing may be warranted based on the lesion appearance and location.

For vulvar ulcers, consider the followings:

  • HSV cell culture or polymerase chain reaction (PCR) from lesion

  • Nontreponemal and treponemal tests for syphilis

  • If both of the above are negative and clinical scenario is appropriate, consider chancroid

  • Giemsa stain for Donovan bodies if granuloma inguinale is in the differential diagnosis

  • Lesion swab or bubo aspirate for Chlamydia trachomatis if lymphogranuloma venereum is in the differential diagnosis

  • Bacterial culture of wound base if decubitus ulcer is present

For the evaluation and diagnosis of cervicitis see the chapter on cervicitis and pelvic inflammatory disease.

C. Criteria for Diagnosing Each Diagnosis in the Method Above.

The criteria for diagnosing underlying systemic diseases such as Crohn's disease, Behcet's syndrome, and pemphigus vulgaris are discussed in the separate chapters on those diseases. General criteria and methods for diagnosis of the extensive differential diagnosis are discussed in the sections above.

D. Over-utilized or “wasted” diagnostic tests associated with the evaluation of this problem.

Vaginal swabs for bacterial culture are rarely, if ever useful as they will likely pick up normal vaginal flora. Imaging of the lower genital tract is also rarely useful as most findings are visible on examination, colposcopy or biopsy.

A. Management of diseases of the external genitalia to cervix

When lower genital tract disease is suspected to be part of an underlying systemic illness such as Behcet's syndrome or Crohn's disease the hospitalist can initiate the appropriate treatment while waiting for confirmation of the diagnosis. The same is true for infectious causes. If the hospitalist is unsure how to interpret exam findings, a gynecology consultation to confirm and possibly biopsy findings may be helpful.

Most of the conditions listed above are not immediately life-threatening. However, in a female patient with unstable vital signs and no clear source for sepsis, or with significant pelvic pain, the hospitalist must consider toxic shock syndrome, pelvic inflammatory disease, cellulitis and necrotizing fasciitis as possible diagnoses. These should be treated with the appropriate antimicrobial therapy, with surgical evaluation or blood pressure support as warranted.

B. Common Pitfalls and Side-Effects of Management of this Clinical Problem

An examination before starting treatment is an essential, though often overlooked step and will usually guide management. If a dermatitis is suspected, attempts should be made to remove irritants. If symptoms are mild, the hospitalist can prescribe low-potency topical steroids. Note, short courses of high potency topical steroids for this and other dermatoses may be warranted and are an acceptable treatment. If gonococcal or chlamydial cervicitis are suspected, then empiric antimicrobial treatment is warranted while confirmatory tests are pending.

IV. What's the evidence?

Augenbraun, MH, Mandell, GL,, Bennett, JE,, Dolin, R. "Genital skin and mucous membrane lesions.". Principles and Practice of Infectious Diseases. 2010. pp. 1484-1475.

Beecker, J. "Therapeutic principles in vulvovaginal dermatology.". Dermatology Clinics. vol. 28. 2010. pp. 639-648.

Casey, PM,, Long, ME,, Marnach, ML. "Abnormal cervical appearance: what to do, when to worry?". Mayo Clinic Proceedings. vol. 86. 2011. pp. 147-151.

Eckert, LO. "Acute vulvovaginitis.". New England Journal of Medicine. vol. 355. 2006. pp. 1244-52.

Foster, DC. "Vulvar disease". Obstetrics and Gynecology. vol. 100.

Foster, DC,, Duecy, E, Duthie, EH,, Katz, PR,, Malone, ML. "Gynecologic disorders". Saunders Elsevier. 2007. pp. 607-615.

Hoffman, BL,, Schorge, JO,, Schaffer, JI,, Halvorson, LM, Hoffman, BL,, Schorge, JO,, Schaffer, JI,, Halvorson, LM,, Bradshaw, KD,, Cunningham, F,, Calver, LE. eds., Williams, Gynecology. "Chapter 4.". Benign Disorders of the Lower Reproductive Tract. McGraw-Hill. 2012.

Katz, VL, Katz, VL,, Lentz, GM,, Lobo, RA,, Gershenson, DM. "Benign gynecologic lesions: vulva, vagina, cervix, uterus, oviduct, ovary.". Comprehensive Gynecology. Mosby Elsevier. 2007. pp. 419-471.

Pipkin, C. "Erosive diseases of the vulva". Dermatology Clinics. vol. 28. 2010. pp. 737-751.

Say, PJ,, Jacyntho, C. "Difficult-to-manage vaginitis". Clinical Obstetrics and Gynecology. vol. 48. 2005. pp. 753-768.

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