eMedicine Specialties > Dermatology > Diseases of the Dermis

Balanitis Xerotica Obliterans

Author: Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Coauthor(s): George C Keough, MD, Chief, Clinical Assistant Professor, Department of Medicine, Dermatology Service, Eisenhower Army Medical Center; Daniel S Lehman, MD, Fellow in Minimally Invasive Urology/Oncology, Department of Urology, Columbia University Medical Center
Contributor Information and Disclosures

Updated: Mar 17, 2008

Introduction

Background

Lichen sclerosus (LS) is a chronic, progressive, sclerosing inflammatory dermatosis of unclear etiology. Most reported LS cases (83%) involve the genitalia. In men, this genital involvement has traditionally been known as balanitis xerotica obliterans (BXO). A more accurate term is male genital or penile LS.

Yardley et al¹ believe that the prevalence of BXO is greater than previous series have shown and that it may manifest in children at an earlier age than previous series have shown. This belief is based on a study of 422 boys at a median age of 6 years 2 months (range, 3 mo to 16 y), of whom 186 (44.1%) received treatment involving surgery (148 circumcision, 33 preputial adhesiolysis, 5 frenuloplasty). Of the 186 boys, 110 had histological tissue examination; 84.8% of skin samples were pathologic. Specifically, tissue showed chronic inflammation (n = 69; 46.6%), BXO (n = 51; 34.5%), and fibrosis (n = 4; 2.7%).

For related eMedicine articles, see Lichen Sclerosus et Atrophicus, Balanitis Circumscripta Plasmacellularis, and Balanitis. Also, Medscape's CME course HPV-Related Disease in Men: Anal and Penile Cancer (Slides With Transcript) may be of interest.

Pathophysiology

The etiology of male genital LS is unknown, but it is thought to be multifactorial. BXO has occurred in monozygotic twins, which suggests a genetic basis for the disease in some cases. Human papillomavirus type 6 or type 16 has not been detected in patients with BXO, which strongly suggests that genital papillomaviruses do not have a strong association with BXO.

Frequency

United States

Kizer et al² noted that of 153,432 male patients discharged from Brooke Army Medical Center, 108 (0.070%) had a diagnosis of BXO. The age distribution was similar over a range of 2-90 years, with the exception of the third decade, when the incidence almost doubled. Black and Hispanic patients had twice the rate found in white patients (10.59 cases, 10.67 cases, and 5.07 cases per 10,000 patients, respectively).

International

The prevalence of male genital LS has traditionally been estimated at 1 case per 300-1000 males. No recent studies confirm this estimate, but male genital LS is not considered a rare condition. Huntley et al³ reported on 100 consecutive patients seen in pediatric urology clinics who were followed to discharge. Eighteen referrals for circumcision were for religious reasons. Of the other 82 patients, the main reason for referral was retractability or phimosis. Six patients were identified as having BXO, a condition that had not been suggested on referral.

Race

Male genital LS has no known predilection for any racial or ethnic group.

Sex

Male genital LS occurs most frequently in persons who are uncircumcised and who are of middle age. One study⁴ revealed that 51 (98%) of 52 patients clinically diagnosed with penile LS were uncircumcised.

Age

Although males with genital LS are most frequently of middle age, the condition also may appear in children, ranging from young boys to adolescents. The incidence of BXO in pediatric patients is higher than most physicians realize. Additionally, the incidence of BXO is high in boys with phimosis.^5,6,7

Clinical

History

Early in its course, penile LS is relatively asymptomatic with only mild visually observable changes of the penis and glans. Physical changes occur over months or years and may include color or textural changes. Early symptoms are more prevalent in uncircumcised patients.

• Symptoms occurring with time and progression of penile LS are as follows:
  • Pruritus
  • Burning
  • Hypoesthesia of the glans penis
  • Dysuria
  • Painful erection with altered sexual function
  • Decrease in urinary force or stream caliber
  • Urethritis with or without discharge
• Symptoms occurring in late penile LS (in uncircumcised patients) are as follows:
  • Phimosis (inability to retract the foreskin over the glans)
  • Paraphimosis (inability to return an already retracted foreskin back over the glans)
• The development of multifocal squamous cell carcinoma (SCC) in persons with LS et atrophicus of the penis and hepatitis C virus infection has been reported. SCC of the penis arising from BXO alone has also been noted.
• A urethral stone manifesting as a stop valve, a rare complication of BXO, has been reported.

Physical

Early penile LS demonstrates only subtle physical findings (eg, mild, nonspecific erythema; mild hypopigmentation).

• As the condition progresses, single or multiple discrete erythematous papules or macules progress and coalesce into atrophic ivory, white, or purple-white patches or plaques.
  • Lesions most commonly affect the glans and prepuce.
  • The frenulum, urethral meatus, fossa navicularis, penile shaft, and perianal areas may become involved.
  • A sclerotic white ring at the tip of the prepuce is diagnostic at this stage.
  • Erosions, fissures, petechiae, serous and hemorrhagic bullae, and telangiectasias of the glans have been reported, albeit uncommonly.
• With further disease progression, the glans may become adherent to the prepuce.
  • The coronal sulcus and frenulum may be sclerotically destroyed.
  • The urethral meatus may narrow to the point of urinary retention.
  • Urinary retention may be severe enough to cause retrograde damage to the posterior urethra and to the bladder and kidneys.
  • Significant urethral meatal narrowing has led to sloughing of the distal half centimeter of the urethra.
  • Phimosis and paraphimosis of uncircumcised patients may occur at this late stage.
• Seventeen percent of LS cases are extragenital, beginning as mild, nonspecific erythema with mild hypopigmentation.
• In one case report⁸ , BXO in a middle-aged man involved the entire anterior urethra and the scrotum. It manifested as a palpable nodular scrotal mass and caused obstructive voiding symptoms. He was treated with a staged urethroplasty.

Causes

The etiology of male genital LS is unknown but is thought to be multifactorial. Several contributing factors are possible, as follows:

• Circumcision after age 13 years/uncircumcised state
  • This may very well be due to the effect known as the isomorphic, or Koebner, phenomenon. The large majority of inflammatory dermatoses of the male genitalia, including LS, occur in uncircumcised or late-circumcised men.
  • The presence of a foreskin may promote chronic irritation or serve to maintain a friendly environment for an as-yet unidentified infectious agent. Such chronic irritation and subsequent inflammation may initiate the changes noted in LS.
• Hormonal factors
  • Hormonal influences in the development of LS have long been postulated, mainly in female vulvar LS.
  • Most studies have concentrated on the role of testosterone in the pathogenesis of vulvar LS. Childhood vulvar LS frequently resolves with the onset of menarche and the related pubertal increase in testosterone production in genital skin; additionally, adults with LS have been found to have decreased serum levels of free testosterone, androstenedione, and dihydrotestosterone compared with control subjects.
  • The underlying defect may be a problem with the function of the enzyme 5-alpha reductase.
• Autoimmune disease
  • Various autoantibodies (including antinuclear, thyroid antimicrosomal, antigastric parietal cell, anti-adrenal cortex, antismooth muscle, and antimitochondrial antibodies) have been detected in patients with LS.
  • Vitiligo, thyroid disease, diabetes, and alopecia areata have also been commonly reported in association with LS.
• Genetic factors
  • LS (not necessarily genital LS) has been reported in families, including twins (identical and nonidentical), sisters, mothers and daughters, and a brother and sister.
  • Note, however, that no consistent pattern of genetic inheritance has been identified.
• Presence of human papillomaviruses
  • The presence of human papillomaviruses (HPV) has been reported in some cases of childhood penile LS. Whether the LS is directly attributable to HPV infection, or if LS merely promotes HPV infection is unclear.
  • Patients with penile LS alone have not been demonstrated to have a higher incidence of HPV infection.
• Other: In a study of 18 patients⁹ with combined buccal mucosa grafting and genital skin flap reconstruction of extensive anterior urethral strictures, 16.7% of stricture cases were caused by BXO.

• Search for CME/CE on This Topic »

• Lichen Sclerosus et Atrophicus (Dermatology)
• Penile Cancer (Urology)
• Dermatologic Diseases of the Male Genitalia (Urology)

• Is Neonatal Circumcision Clinically Beneficial? Argument Against