Balanitis xerotica obliterans

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Vishal Devarkonda, M.B.B.S[2]

Synonyms and keywords:BXO, Penile lichen sclerosus

Overview

Balanitis xerotica obliterans (BXO) is a dermatological (skin) condition affecting the male genitalia. It was first described by Stuhmer in 1928, though earlier reports describe what may have been the same condition. The exact pathophysiology of Balanitis xerotica obliterans is unknown. BXO commonly occurs on the foreskin and glans penis. Patients with BXO usually present with atrophic white patches with whitish ring of indurated (hardened) tissue near the tip that may prevent retraction of skin on penis. Definitive diagnosis is provided by cutaneous biopsy. Treatment options include both Medical and surgical modalities.

Historical Perspective

In 1928, Stuhmer for the first time in medical literature described lichen sclerosus as Balanitits Xerotica obliterans

Classification

There is no established classification system for BXO.

Pathophysiology

The exact pathophysiology of BXO is unknown, but multiple factors are considered to play an important in the development of BXO. These include:[1][2][3][4][5][6][7][8]

Factors associated with pathogenesis of BXO
Uncircumcised Penis Accumulation of secretions and epithelial debris between the foreskin and coronal sulcus leads to chronic irritation, sublincal trauma. [1]
Autoimmune diseases Patients with BXO, were found to have an other associated autoimmune conditions, which include: diabetes mellitus, vitiligo, alopecia aerata.[3]

Some studies have showned association between BXO and HLA DQ7 with DR11 and DR12.[2]

Infections Human papillomavirus (HPV) Several studies have implicated human papillomavirus as a causative agent in pathogenesis of BXO. HPV 16, 18, 33 and 51 have been found to associated with BXO.

Recent studies reported lack of clincal correlation of BXO and HPV, has they both have unrelated transcriptosome.

Several studies have reported association of various infectious organisms with development of Balanitis xerotica obliterans, which include:
  • Borrelia burgdoferi[4]
  • HCV[5]
  • Epstein-Barr virus[6]
Genetics Several studies have proposed genetic association and lichen sclerosis.

In females, 12% of patients were found to have a family history of lichen sclerosis,.

In males, there is no evidence familial predisposition.

Environmental factors

BXO is known to demonstrate koebner phenomenon.[7]

Trauma, old scars, skin grafts, sunburn and radiation were found to be associated with BXO.[7]

Some studies have proposed that post-micturation dribbling or microincontinence plays a central role in development of BXO.[8]

Histopathology

Histopatholgy findings found in BXO include:[9]

Early stage of BXO

  • Moderately heavy lymphocytic infiltrate in found in basal epidermis and superficial dermis in early stages of the lesion.

Late stages of BXO

  • Epidermis becomes atrophic with surface hyperkeratosis, thickened basement membrane
  • Broad zone of subepidermal oedema with homogenization of collagen, which becomes more sclerotic over time.
  • In few cases, epidermis is detached from dermis resulting in formation of haemorrhagic bullae.
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Epidemiology and Demographics

Incidence

The true incidence and prevalence of BXO remains unclear.

A study as reported incidence at 70/100,00(0.07%, In an unselected cohort of 153 432 patients presenting to an outpatient clinic in Brookes Army Medical Centre in the USA)

Age

BXO commonly affects middle age group, with men in their twenties were at twice the risk.[10]

Race

On comparison with white men, BXO is more prevalent in black and hispanic men.[11]

Screening

There is no established screening guidelines for BXO.

Natural History, Complications, and Prognosis

Natural history

If left untreated, BXO involve the penile skin, scrotum, and entire urethra leading to the complications such as phimosis and urinary retention.[12]

Complications

Complication of BXO include the following:[13][14][15][16][17][18]

  • Phimosis
  • Painful erection
  • Reduced urinary flow
  • Urinary retention
  • Risk of malignant transformation into Squamous cell carcinoma(Long-term prospective studies are needed to determine the real risk of malignant transformation)

Prognosis

Prognosis is good with treatment.[19]

Diagnosis

History and symptoms

Patients with BXO could be asymptomatic or present with:[20]

  • Whitening or reddening of the penile region
  • Difficulty in retracting the foreskin
  • Painful erection
  • Reduced urinary flow
  • Urinary retention
  • Buring sensation ( paraesthesia)(rare)
  • purple rash in gential region( purpura)(rare)
  • Small red or purple clusters, often spidery in appearance, on penis(telangiectases)(rare)
  • Itching (pruritus) of the genitalia(rare)
  • Discomfort in urination(dysuria)(rare)

Physical examination

Physical examination findings include:[21]

Laboratory findings

Cutaneous biopsy, along with a rapid protein reagin test, will provide a definitive diagnosis.[22]

Cutaneous biopsy

Treatment

Treatment of BXO include both surgical and medical modalities, these include:[23][24][25][26][27][26][28][29]

Managements of BXO
Medical Drug dosage Effectiveness
Topical steroids Betamethasone diproprionate 0.05% or clobetasol proprionate 0.05% cream or ointment once or twice daily

After 6–8 weeks, reduce the application of the topical steroid to every second day

After 12–16 weeks to assess response to treatment(mometasone aceponate 0.1% cream can be substituted if there is a good response)

No improvement by 6 months, then use of the potent topical steroid should be abandoned.

Studies have shown that 50% of patients respond to topical steroid application.
Topical calcineurin inhibitors Tacrolimus ointment 0.1% twice daily Shouldn't be used as first-line therapy
Pimecrolimus cream 1% twice daily
Tricyclic antidepressant or gabapentin Can be used in cases when BXO is associated with penile dysesthesia.

Surgery

  • Surgical treatment often involves circumcision. Trial of steroids is usually prescribed before subjecting patients for surgery. Phimosis is an indication for surgery. In patients with severe BXO may require an extensive surgery with disease control, function and cosmesis in carefully balanced.
  • Some cases may require meatoplasty, extensive urethroplasty and reconstructions.
  • Patients who undergo surgery should be follow up as the disease as tendency to recur(BXO as high tendency to recur due to koebner phenomenon).
  • Patients should be advice for regular genital self-examination and should be advice to return if the lesion recur.

Prospective therapies

Intralesional corticosteroids, topical and intramuscular testosterone, intravenous procaine, topical oestrogen and retinoid creams, oral vitamin E, radiation therapy and CO2 laser are currently been studies for there role in treating BXO.

Prevention

There is no known means of preventing BXO.

Primary Prevention

Circumcision in males can help in reducing risk of having BXO.[30]

Secondary prevention

There are no secondary preventive measures .

References

  1. ↑ 1.0 1.1 Schempp C, Bocklage H, Lange R, Kölmel HW, Orfanos CE, Gollnick H (1993). "Further evidence for Borrelia burgdorferi infection in morphea and lichen sclerosus et atrophicus confirmed by DNA amplification". J Invest Dermatol. 100 (5): 717–20. PMID 8491994.
  2. ↑ 2.0 2.1 Azurdia RM, Luzzi GA, Byren I, Welsh K, Wojnarowska F, Marren P; et al. (1999). "Lichen sclerosus in adult men: a study of HLA associations and susceptibility to autoimmune disease". Br J Dermatol. 140 (1): 79–83. PMID 10215772.
  3. ↑ 3.0 3.1 Meffert JJ, Davis BM, Grimwood RE (1995). "Lichen sclerosus". J Am Acad Dermatol. 32 (3): 393–416, quiz 417-8. PMID 7868709.
  4. ↑ 4.0 4.1 Fujiwara H, Fujiwara K, Hashimoto K, Mehregan AH, Schaumburg-Lever G, Lange R; et al. (1997). "Detection of Borrelia burgdorferi DNA (B garinii or B afzelii) in morphea and lichen sclerosus et atrophicus tissues of German and Japanese but not of US patients". Arch Dermatol. 133 (1): 41–4. PMID 9006371.
  5. ↑ 5.0 5.1 Boulinguez S, Bernard P, Lacour JP, Nicot T, Bedane C, Ortonne JP; et al. (1997). "Bullous lichen sclerosus with chronic hepatitis C virus infection". Br J Dermatol. 137 (3): 474–5. PMID 9349358.
  6. ↑ 6.0 6.1 Aidé S, Lattario FR, Almeida G, do Val IC, da Costa Carvalho M (2010). "Epstein-Barr virus and human papillomavirus infection in vulvar lichen sclerosus". J Low Genit Tract Dis. 14 (4): 319–22. doi:10.1097/LGT.0b013e3181d734f1. PMID 20885159.
  7. ↑ 7.0 7.1 7.2 Bjekić M, Šipetić S, Marinković J (2011). "Risk factors for genital lichen sclerosus in men". Br J Dermatol. 164 (2): 325–9. doi:10.1111/j.1365-2133.2010.10091.x. PMID 20973765.
  8. ↑ 8.0 8.1 Bunker CB (2007). "Male genital lichen sclerosus and tacrolimus". Br J Dermatol. 157 (5): 1079–80. doi:10.1111/j.1365-2133.2007.08179.x. PMID 17854373.
  9. ↑ Clouston D, Hall A, Lawrentschuk N (2011). "Penile lichen sclerosus (balanitis xerotica obliterans)". BJU Int. 108 Suppl 2: 14–9. doi:10.1111/j.1464-410X.2011.10699.x. PMID 22085120.
  10. ↑ Kizer WS, Prarie T, Morey AF (2003). "Balanitis xerotica obliterans: epidemiologic distribution in an equal access health care system". South Med J. 96 (1): 9–11. PMID 12602705.
  11. ↑ Kizer WS, Prarie T, Morey AF (2003). "Balanitis xerotica obliterans: epidemiologic distribution in an equal access health care system". South Med J. 96 (1): 9–11. PMID 12602705.
  12. ↑ Depasquale I, Park AJ, Bracka A (2000). "The treatment of balanitis xerotica obliterans". BJU Int. 86 (4): 459–65. PMID 10971272.
  13. ↑ Neill SM, Lewis FM, Tatnall FM, Cox NH, British Association of Dermatologists (2010). "British Association of Dermatologists' guidelines for the management of lichen sclerosus 2010". Br J Dermatol. 163 (4): 672–82. doi:10.1111/j.1365-2133.2010.09997.x. PMID 20854400.
  14. ↑ Nasca MR, Innocenzi D, Micali G (1999). "Penile cancer among patients with genital lichen sclerosus". J Am Acad Dermatol. 41 (6): 911–4. PMID 10570372.
  15. ↑ Velazquez EF, Cubilla AL (2003). "Lichen sclerosus in 68 patients with squamous cell carcinoma of the penis: frequent atypias and correlation with special carcinoma variants suggests a precancerous role". Am J Surg Pathol. 27 (11): 1448–53. PMID 14576478.
  16. ↑ Prowse DM, Ktori EN, Chandrasekaran D, Prapa A, Baithun S (2008). "Human papillomavirus-associated increase in p16INK4A expression in penile lichen sclerosus and squamous cell carcinoma". Br J Dermatol. 158 (2): 261–5. doi:10.1111/j.1365-2133.2007.08305.x. PMC 2268980. PMID 18047520.
  17. ↑ Thami GP, Kaur S (2003). "Genital lichen sclerosus, squamous cell carcinoma and circumcision". Br J Dermatol. 148 (5): 1083–4. PMID 12786863.
  18. ↑ Ranjan N, Singh SK (2008). "Malignant transformation of penile lichen sclerosus: exactly how common is it?". Int J Dermatol. 47 (12): 1308–9. doi:10.1111/j.1365-4632.2008.03866.x. PMID 19126024.
  19. ↑ Clouston D, Hall A, Lawrentschuk N (2011). "Penile lichen sclerosus (balanitis xerotica obliterans)". BJU Int. 108 Suppl 2: 14–9. doi:10.1111/j.1464-410X.2011.10699.x. PMID 22085120.
  20. ↑ Clouston D, Hall A, Lawrentschuk N (2011). "Penile lichen sclerosus (balanitis xerotica obliterans)". BJU Int. 108 Suppl 2: 14–9. doi:10.1111/j.1464-410X.2011.10699.x. PMID 22085120.
  21. ↑ Clouston D, Hall A, Lawrentschuk N (2011). "Penile lichen sclerosus (balanitis xerotica obliterans)". BJU Int. 108 Suppl 2: 14–9. doi:10.1111/j.1464-410X.2011.10699.x. PMID 22085120.
  22. ↑ Das S, Tunuguntla HS (2000). "Balanitis xerotica obliterans--a review". World J Urol. 18 (6): 382–7. doi:10.1007/PL00007083. PMID 11204255.
  23. ↑ Clouston D, Hall A, Lawrentschuk N (2011). "Penile lichen sclerosus (balanitis xerotica obliterans)". BJU Int. 108 Suppl 2: 14–9. doi:10.1111/j.1464-410X.2011.10699.x. PMID 22085120.
  24. ↑ Sagi L, Trau H (2011). "The Koebner phenomenon". Clin Dermatol. 29 (2): 231–6. doi:10.1016/j.clindermatol.2010.09.014. PMID 21396563.
  25. ↑ Das S, Tunuguntla HS (2000). "Balanitis xerotica obliterans--a review". World J Urol. 18 (6): 382–7. doi:10.1007/PL00007083. PMID 11204255.
  26. ↑ 26.0 26.1 Hrebinko RL (1996). "Circumferential laser vaporization for severe meatal stenosis secondary to balanitis xerotica obliterans". J Urol. 156 (5): 1735–6. PMID 8863582.
  27. ↑ Rudolph R, Walther P (1997). "Full-thickness skin grafts from eyelids to penis, plus split-thickness grafts in chronic balanitis xerotica obliterans". Ann Plast Surg. 38 (2): 173–6. PMID 9043588.
  28. ↑ Singh I, Ansari MS (2006). "Extensive balanitis xerotica obliterans (BXO) involving the anterior urethra and scrotum". Int Urol Nephrol. 38 (3–4): 505–6. doi:10.1007/s11255-006-0100-8. PMID 17180441.
  29. ↑ Garaffa G, Shabbir M, Christopher N, Minhas S, Ralph DJ (2011). "The surgical management of lichen sclerosus of the glans penis: our experience and review of the literature". J Sex Med. 8 (4): 1246–53. doi:10.1111/j.1743-6109.2010.02165.x. PMID 21210959.
  30. ↑ Dayal S, Sahu P (2016). "Zoon balanitis: A comprehensive review". Indian J Sex Transm Dis. 37 (2): 129–138. doi:10.4103/0253-7184.192128. PMC 5111296. PMID 27890945.

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