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Balanitis Xerotica Obliterans Clinical Presentation

  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: William D James, MD  more...
 
Updated: Feb 12, 2016
 

History

Early in its course, penile lichen sclerosus (balanitis xerotica obliterans [BXO]) 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 lichen sclerosus 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 lichen sclerosus (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 lichen sclerosus 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.

In older patients, BXO with phimosis can be a cause of difficulty with urination; thus, older patients should be examined to see if they have BXO in they have symptoms of difficulty with urination.[12]

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Physical

Early penile lichen sclerosus (balanitis xerotica obliterans [BXO]) 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 lichen sclerosus cases are extragenital, beginning as mild, nonspecific erythema with mild hypopigmentation.

In one case report[13] , 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.

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Causes

The etiology of male genital lichen sclerosus (balanitis xerotica obliterans [BXO]) is unknown but is thought to be multifactorial. Several contributing factors are possible, as described below.

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 lichen sclerosus, 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 lichen sclerosus.

Hormonal factors

Hormonal influences in the development of lichen sclerosus have long been postulated, mainly in female vulvar lichen sclerosus.

Most studies have concentrated on the role of testosterone in the pathogenesis of vulvar lichen sclerosus. Childhood vulvar lichen sclerosus frequently resolves with the onset of menarche and the related pubertal increase in testosterone production in genital skin; additionally, adults with lichen sclerosus 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 lichen sclerosus.

Vitiligo, thyroid disease, diabetes, and alopecia areata have also been commonly reported in association with lichen sclerosus.

Genetic factors [14]

Lichen sclerosus (not necessarily genital lichen sclerosus) 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 lichen sclerosus. Whether the lichen sclerosus is directly attributable to HPV infection, or if lichen sclerosus merely promotes HPV infection is unclear.

Patients with penile lichen sclerosus alone have not been demonstrated to have a higher incidence of HPV infection.

Other

In a study of 18 patients[15] with combined buccal mucosa grafting and genital skin flap reconstruction of extensive anterior urethral strictures, 16.7% of stricture cases were caused by BXO.

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Contributor Information and Disclosures
Author

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie<br/>Received income in an amount equal to or greater than $250 from: Optigenex<br/>Received salary from Optigenex for employment.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD Associate Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Mark W Cobb, MD Consulting Staff, WNC Dermatological Associates

Mark W Cobb, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology

Disclosure: Nothing to disclose.

Acknowledgements

George C Keough, MD Chief, Clinical Assistant Professor, Department of Medicine, Dermatology Service, Eisenhower Army Medical Center

George C Keough, MD is a member of the following medical societies: American Academy of Dermatology and American Medical Association

Disclosure: Nothing to disclose.

Daniel S Lehman, MD Fellow in Minimally Invasive Urology/Oncology, Department of Urology, Columbia University Medical Center

Disclosure: Nothing to disclose.

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Balanitis xerotica obliterans (lichen sclerosus). Courtesy of Wilford Hall Medical Center Slide collection.
 
 
 
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