Continuing Education Activity

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

Objectives:

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

Introduction

Balanitis xerotica obliterans, also known as lichen sclerosus, are white inflammatory patches affecting 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 the prevalence of 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 are most likely affected, with a mean incidence of 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 is 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, 1 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 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 are white or erythematous areas on the glans, penis, or foreskin. Phimosis may be seen as the condition progresses. Mattioli et al. showed 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 be present on the penis. Contact dermatitis can arise anywhere on the boys and is 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 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 results from 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 2 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

The differential diagnosis for balanitis xerotica obliterans includes the following:

• 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 10-year retrospective study of patients with balanitis xerotica obliterans. They noted 62.6% had foreskin scarring, 47.2% of patients had foreskin and meatus involvement, and 26.4% had foreskin, glans, and meatus involvement. Of their patient population, the majority required circumcision before age sixteen, and an additional 19.8% of patients needed an additional procedure following their circumcision 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 is 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 are used for symptomatic patients. Surgery is 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 primary care physicians and 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 scheduled for further treatment.

Review Questions

• Access free multiple choice questions on this topic.
• Comment on this article.

References

1.

Nguyen ATM, Holland AJA. Balanitis xerotica obliterans: an update for clinicians. Eur J Pediatr. 2020 Jan;179(1):9-16. [PubMed: 31760506]

2.

Das S, Tunuguntla HS. Balanitis xerotica obliterans--a review. World J Urol. 2000 Dec;18(6):382-7. [PubMed: 11204255]

3.

Boksh K, Patwardhan N. Balanitis xerotica obliterans: has its diagnostic accuracy improved with time? JRSM Open. 2017 Jun;8(6):2054270417692731. [PMC free article: PMC5464383] [PubMed: 28620502]

4.

Celis S, Reed F, Murphy F, Adams S, Gillick J, Abdelhafeez AH, Lopez PJ. Balanitis xerotica obliterans in children and adolescents: a literature review and clinical series. J Pediatr Urol. 2014 Feb;10(1):34-9. [PubMed: 24295833]

5.

Clouston D, Hall A, Lawrentschuk N. Penile lichen sclerosus (balanitis xerotica obliterans). BJU Int. 2011 Nov;108 Suppl 2:14-9. [PubMed: 22085120]

6.

Fergus KB, Lee AW, Baradaran N, Cohen AJ, Stohr BA, Erickson BA, Mmonu NA, Breyer BN. Pathophysiology, Clinical Manifestations, and Treatment of Lichen Sclerosus: A Systematic Review. Urology. 2020 Jan;135:11-19. [PubMed: 31605681]

7.

Buechner SA. Common skin disorders of the penis. BJU Int. 2002 Sep;90(5):498-506. [PubMed: 12175386]

8.

Folaranmi SE, Corbett HJ, Losty PD. Does application of topical steroids for lichen sclerosus (balanitis xerotica obliterans) affect the rate of circumcision? A systematic review. J Pediatr Surg. 2018 Nov;53(11):2225-2227. [PubMed: 29395150]

9.

Pradhan A, Patel R, Said AJ, Upadhyaya M. 10 Years' Experience in Balanitis Xerotica Obliterans: A Single-Institution Study. Eur J Pediatr Surg. 2019 Jun;29(3):302-306. [PubMed: 30130825]

10.

Philippou P, Shabbir M, Ralph DJ, Malone P, Nigam R, Freeman A, Muneer A, Minhas S. Genital lichen sclerosus/balanitis xerotica obliterans in men with penile carcinoma: a critical analysis. BJU Int. 2013 May;111(6):970-6. [PubMed: 23356463]

Disclosure: Kevin Carocci declares no relevant financial relationships with ineligible companies.

Disclosure: Gregory McIntosh declares no relevant financial relationships with ineligible companies.