Continuing Education Activity

Balanitis xerotica obliterans, also known as lichen sclerosus, are white inflammatory patches that can affect both males and females. In males, the affected areas usually involve the foreskin and penile glans, termed specifically balanitis xerotica obliterans. This activity describes the evaluation of balanitis xerotica obliterans and highlights the salient features in managing patients with this condition.

Objectives:

• Identify the etiology of balanitis xerotica obliterans.
• Review the appropriate evaluation of balanitis xerotica obliterans.
• Outline the management options available for balanitis xerotica obliterans.
• Explain the importance of improving care coordination amongst the interdisciplinary team to enhance care delivery for patients affected by balanitis xerotica obliterans and improve outcomes.

Introduction

Balanitis xerotica obliterans, also known as lichen sclerosus, are white inflammatory patches that can affect both males and females. In males, the affected areas usually involve the foreskin and penile glans, termed specifically balanitis xerotica obliterans. Urethral stricture disease and meatal stenosis are common sequelae of this condition.[1][2]

Etiology

The etiology of balanitis xerotica obliterans is unknown. It has been proposed that various infections, trauma to the penis, or chronic inflammatory states can lead to this condition.[3]

Epidemiology

Reporting on prevalence regarding this condition is challenging, with an initial presentation to a wide array of physician specialties. Studies have reported prevalence rates surrounding lichen sclerosus, agreeing on approximately 0.1 to 0.3 percent.[3] 

Males 6.8 to 9 years old are most likely affected, with a mean incidence at age 7. Lack of circumcision has been reported as a risk factor for developing balanitis xerotica obliterans. Several studies have shown no race predilection; however, Nguyen et al. report higher rates in African American and Hispanic populations likely secondary to lower rates of circumcision.[1]

Pathophysiology

The exact etiology is unknown. However, growing evidence suggests autoimmune influences, a possible genetic predisposition, and inflammatory insults leading to disease progression.[4]

History and Physical

Most often, balanitis will be found in uncircumcised men or older men who have had a circumcision later in life. Again, the etiology is unknown, but chronic inflammation and irritation seem to be the most notable inciting event for disease progression. Balanitis is oftentimes initially presenting asymptomatic. However, one of the more common physical exam findings would be erythematous changes or white hypopigmented lesions seen on the glans penis or foreskin.[5] 

This generalized inflammation can lead to a condition known as phimosis. Phimosis refers to difficulty retracting the foreskin and can lead to challenges with micturition and overall sexual function. If left unattended, additional symptoms can develop, including dysuria, urinary retention, and renal failure. The erythematous changes can further develop into more defined lesions around the coronal sulcus and foreskin. Eventually, this can progressively invade the urethral meatus and fossa navicularis, affecting the urethral tract, and strictures disease, or narrowing, can result.

Evaluation

Balanitis is usually seen in uncircumcised males. There will be white or erythematous areas on the glans penis or foreskin. Phimosis may be seen as the condition progresses. Mattioli et al. show in their study that 15% of the foreskins studied showed hypospadias.[4] The differential diagnosis includes psoriasis, neoplastic process, contact dermatitis, Zoon balanitis, leukoplakia, and fixed drug reaction.

Malignant transformation to squamous cell carcinoma occurs in 3% to 6% of females and 2% to 8% of males.[6]

Psoriasis is a chronic disease with red pruritic patches that can arise anywhere on the body. This condition is not contagious. It is an immune condition where skin cells turn over at an increased rate. Plaques are commonly seen and can present on the penis.

Contact dermatitis can arise anywhere on the boys and presents as a pruritic, erythematous area caused by contact with a particular substance. The affected area can look like a red rash with cracked skin.

Plasma cell balanitis, or Zoon balanitis, is a benign condition that usually presents in older men. The patient usually presents with a flat red plaque that sometimes has associated smaller red marks. Diagnosis requires a biopsy.[7]

Leukoplakia can present very similarly to lichen sclerosis, where the plaque is white and flat. There can be a malignant transformation in this condition. Typically, it is the result of chronic irritation between the glans and foreskin.

Lichen sclerosus can be diagnosed based on clinical findings. A biopsy can be performed to rule out differential causes of symptoms. In patients who have lesions in the urethral, a cystoscopy to directly visualize the extent of the disease is warranted.

Treatment / Management

There are both medical and surgical options for lichen sclerosus of the penis. Asymptomatic balanitis xerotica obliterans does not require therapy. For symptomatic lichen sclerosus, topical steroids are the main treatment. Betamethasone and triamcinolone are common options and typically require twice a day dosing. The patient is monitored for a response, and the frequency of topical steroids can be reduced to every other day or every third day if a good response after two months.[8] 

Poor response is typically seen when the patient is not compliant with the application. Topical calcineurin inhibitors have also been used but are considered second-line therapy. These include pimecrolimus and tacrolimus. There is no benefit to systemic oral steroids in the treatment.[5]

Surgical options are required in the setting of symptomatic phimosis. The treatment is circumcision. Topical steroid use first may help reduce the inflammatory burden before surgical intervention.

When the urethra is involved, cystourethroscopy can be performed to identify the location of the disease. Direct visualization is important to identify the severity of stricture disease related to lichen sclerosus and exclude other causes of symptoms. Treatment for stricture may involve dilation and direct visual internal urethrotomy, or in more advanced cases or previous treatment failure, urethroplasty may be warranted.

Patients should be followed yearly for disease recurrence or evidence of progression to squamous cell carcinoma.

Differential Diagnosis

• Carcinoma in situ 
• Psoriasis
• Squamous cell carcinoma 
• Cellulitis
• Contact dermatitis 
• Zoon balanitis
• Leukoplakia
• Scleroderma 
• Fixed drug reaction 

Prognosis

Long-term follow-up is necessary given the possibility of progression to stricture disease or carcinoma. Pradhan et al. published a ten-year retrospective study of patients with balanitis xerotica obliterans. They noted 62.6% had foreskin scarring and 47.2% of patients had foreskin and meatus involvement, and 26.4% had a foreskin, glans, and meatus involvement. Of their patient population, the majority required circumcision before age sixteen, and an additional 19.8% of patients needed ad additional procedure following their circumcision and/or meatal dilation.[9] Early diagnosis and treatment can help prevent the progression of the disease.

Complications

Complications usually arise from late diagnosis and can include phimosis and urinary retention. It has been proposed that chronic inflammation from lichen sclerosus can lead to squamous cell carcinoma. A study out of Italy by Nasca et al. demonstrates that 9.3% of study patients developed penile cancer.[5] Therefore, these studies explain that the association between balanitis xerotica obliterans and penile cancer is underestimated.[10]

Deterrence and Patient Education

Patients should be evaluated yearly by primary care providers. If there is concern about anogenital disorders, proper referral to urologists should be undertaken. Patients should be counseled on proper self-genital exams. If patients are prescribed topical steroids for lichen sclerosus, strict adherence to application schedules must be maintained. There will be a subset of patients requiring more complicated intervention when topical steroids and circumcision fail to correct symptoms.

Enhancing Healthcare Team Outcomes

Balanitis xerotica obliterans is a condition that requires the interprofessional contributions of primary care physicians, nurses, pharmacists, and consulting services, including dermatology and urology. Prescription topical corticosteroids will be used for symptomatic patients. Surgery will be required in a large percentage of patients who continue to have symptoms despite topical therapy. Circumcision and the need for additional surgical intervention may be required.

Follow up with both primary care physicians and the consulting services is important to prevent further progression of inflammatory changes to squamous cell cancer or evidence of urinary difficulty from stricture disease. Clinicians should prepare patients for signs and symptoms to watch for, and when disease progressions occur, a urology consultation should be placed for further treatment.