Lichen sclerosus

Lichen sclerosus

Micrograph of lichen sclerosus showing the characteristic subepithelial sclerosus (right/bottom of image). H&E stain.
Classification and external resources
Specialty dermatology
ICD-10 L90.0
ICD-9-CM 701.0
eMedicine derm/234
MeSH D018459

Lichen sclerosus (LS), and also known as lichen sclerosus et atrophicus (LSetA) [also termed, incorrectly, "Lichen sclerosIs et atrophicus"[1]:227] is a disease of unknown cause that results in white patches on the skin, which may cause scarring on and around genital or sometimes other skin.[2] There is a bimodal age distribution in the incidence of LS. It occurs in females with an average age of diagnosis of 7.6 years in girls and 60 years old in women. The average age of diagnosis in boys is 9–11 years old.[3]

Several risk factors have been proposed, including autoimmune diseases, infections and genetic predisposition.[4][5] There is evidence that LS can be associated with thyroid disease.[6]

Signs and symptoms

Lichen sclerosus on an 82-year-old woman, showing an ivory white coloring in the vulva, and also stretching downward to the perineum.

Women are more commonly affected than men (10 to 1 ratio), particularly around and after menopause, but younger women or girls may also develop the disease. The condition most commonly occurs on the vulva and around the anus with ivory-white elevations that may be flat and glistening. There may be marked itching or the condition may be without any symptoms. There may also be thinning and shrinkage of the genital area that may make coitus, urination, and defecation painful.

In males, the disease may take the form of whitish thickening of the foreskin, which cannot be retracted easily (phimosis). In contrast to women, there is no perianal involvement. In men, this genital involvement has traditionally been known as balanitis xerotica obliterans (BXO).[7]

Only 6% of LS are isolated extragenital lesions.[8] On the non-genital skin, the disease may manifest as porcelain-white spots with small visible plugs inside the orifices of hair follicles or sweat glands on the surface. Thinning of the skin may also occur.[9] [The appearance of the skin, as symptoms listed here, do not include those that may appear in members of some minority groups; I.E. "African-Americans", or others whose skin contains more melanin, may display skin symptoms that appear different than those described here, which would seem to be those that "whites" or Caucasians would most likely display.{Article may need editing to include symptoms displayed by a more diverse cross-section of suffers.}]

Pathophysiology

Although it is not clear what causes LS, several theories have been postulated. Lichen Sclerosus is not contagious; it cannot be caught from another person.[10]

Genetic

Lichen sclerosus may have a genetic component. Higher rates of lichen sclerosus have been reported among twins[11][12] and among family members.[13]

Autoimmunity

Autoimmunity is a process in which the body fails to recognize itself and therefore attacks its own cells and tissue. Specific antibodies have been found in LS. Furthermore, there seems to be a higher prevalence of other autoimmune diseases such as diabetes mellitus type 1, vitiligo and thyroid disease.[14]

Infection

Both bacterial as well as viral pathogens have been implicated in the etiology of LS. A disease that is similar to LS, acrodermatitis chronica atrophicans is caused by the spirochete Borrelia burgdorferi. Viral involvement of HPV[15] and hepatitis C[16] are also suspected.

A link with Lyme Disease is shown by the presence of Borrelia burgdorferi in LSA biopsy tissue.[17]

Hormones

Since LS in females is primarily found in women with a low estrogen state (prepubertal and postmenopausal women), hormonal influences were postulated. To date though, very little evidence has been found to support this theory.

Local skin changes

Some findings suggest that LS can be initiated through scarring[18] or radiation,[19][20] although these findings were sporadic and very uncommon.

Diagnosis

The disease often goes undiagnosed for several years, as it is sometimes not recognized and misdiagnosed as thrush or other problems and not correctly diagnosed until the patient is referred to a specialist when the problem does not clear up.

A biopsy of the affected skin is often done to confirm diagnosis. When a biopsy is done, hyperkeratosis, atrophic epidermis, sclerosis of dermis and lymphocyte activity in dermis are histological findings associated with LS.[21] The biopsies are also checked for signs of dysplasia.[22]

Treatment

There is no definitive cure for LS.[23] Behavior change, such as good hygiene and minimizing scratching of the affected area, is an important part of treatment.[24] LS is also usually treated with potent topical steroids, like clobetasol propionate or mometasone furoate.[23] These can relieve symptoms and prevent scarring.[25] However, LS is a chronic disease so topical steroids may need to be continued as maintenance therapy.[24]

In cases of prepubertal LS, there is some evidence that patients can undergo remission of LS. In a cohort of 12 girls who followed up for 10 years until adolescence, 25% underwent complete remission. Unfortunately, 75% remained symptomatic and demonstrated physical signs of LS into adolescence.[26]

Other treatments including topical hormonal therapies (testosterone and progesterone) have been proposed but not conclusively proven to improve symptoms.[24] Another small study has shown long-term antibiotic treatment to be effective in patients who had poor response to steroids.[22]

It is not considered beneficial to remove LS-affected skin that is not located on the genitals, as it also tends to relapse.

In females, recent studies indicate that the injection of PRP (Platelet-rich plasma) and stem cells in site may reduce symptoms and improve lesions. The usefulness of this treatment in males is under study.[27]

In severe cases, chronic lichen sclerosus may cause anatomical changes, such as labial adhesions or vaginal agglutination, as a result of long-standing inflammation; surgery may be required in these instances.[24]

Recent studies indicate a role for topical calcineurin inhibitors such as tacrolimus.[28][29]

Psychological effect

Distress due to the discomfort and pain of Lichen Sclerosus is normal, as are concerns with self-esteem and sex. Counseling can help. Patients suffering from painful intercourse may also benefit from using lubricants or moisturizers.[24]

According to the National Vulvodynia Association, which also supports women with Lichen Sclerosus, vulvo-vaginal conditions can cause feelings of isolation, hopelessness, low self-image, and much more. Some women are unable to continue working or have sexual relations, and may be limited in other physical activities.[30] Depression, anxiety, and even anger are all normal responses to the ongoing pain LS patients suffer from.

Prognosis

The disease can last for a considerably long time. Occasionally, "spontaneous cure" may ensue,[31] particularly in young girls.

Lichen sclerosus is associated with a higher risk of cancer.[32][33][34] Skin that has been scarred as a result of lichen sclerosus is more likely to develop skin cancer. Women with lichen sclerosus may develop vulvar carcinoma.[35] Lichen sclerosus is associated with 3–7% of all cases of vulvar squamous cell carcinoma.[36] Periodic consultation is therefore necessary.

History

Lichen sclerosus (LS) is also known as lichen sclerosus et atrophicus (LSA), balanitis xerotica obliterans (BXO), Csillag's disease, Lichen albus, Hypoplastic dystrophy, White Spot Disease and kraurosis vulvae. Typically it's called LSA or BXO when it affects men, LS when it affects women or in referring to the disease in general, and pediatric lichen sclerosus when it affects children. LS is usually found in the groin area, but sometimes on the upper leg or thigh.

Lichen sclerosus et atrophicus was first described in 1887 by Dr. Hallopeau.[37] Since not all cases of lichen sclerosus exhibit atrophic tissue, et atrophicus was dropped in 1976 by the International Society for the Study of Vulvovaginal Disease (ISSVD), officially proclaiming the name lichen sclerosus.[38]

See also

References

  1. ↑ James, William D.; Berger, Timothy G. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
  2. ↑ Pugliese, JM; Morey, AF; Peterson, AC (2007). "Lichen Sclerosus: Review of the Literature and Current Recommendations for Management". J Urol 178 (6): 2268–2276. doi:10.1016/j.juro.2007.08.024. PMID 17936829.
  3. ↑ Fistarol, Susanna K (2013). "Diagnosis and treatment of lichen sclerosus: an update.". Journal of American Academy of Dermatology 14 (1): 27–47. doi:10.1007/s40257-012-0006-4.
  4. ↑ Yesudian, PD; Sugunendran, H; Bates, CM; O'Mahony, C (2005). "Lichen sclerosus". Int J STD AIDS 16 (7): 465–473. doi:10.1258/0956462054308440. PMID 16004624.
  5. ↑ Regauer, S (2005). "Immune dysregulation in lichen sclerosus". Eur J Cell Biol 84 (2–3): 273–277. doi:10.1016/j.ejcb.2004.12.003. PMID 15819407.
  6. ↑ Birenbaum, DL; Young, RC (2007). "High prevalence of thyroid disease in patients with lichen sclerosus". J Reprod Med 52 (1): 28–30. PMID 17286064.
  7. ↑ Balanitis Xerotica Obliterans at eMedicine
  8. ↑ Wallace, HJ (1971). "Lichen sclerosus et atrophicus.". Trans St Johns Hosp Dermatol Soc. 57: 9–30.
  9. ↑ Laymon, CW (1951). "Lichen sclerosus et atrophicus and related disorders". MA Arch Derm Syphilol 64 (5): 620–627. doi:10.1001/archderm.1951.01570110090013. PMID 14867888.
  10. ↑ National Institute of Health. "Fast Facts About Lichen Sclerosus". Lichen Sclerosus. National Institute of Arthritis and Musculoskeletal and Skin Diseases. Retrieved 16 June 2012.
  11. ↑ Meyrick Thomas, RH; Kennedy, CT (Mar 1986). "The development of lichen sclerosus et atrophicus in monozygotic twin girls.". The British journal of dermatology 114 (3): 377–379. doi:10.1111/j.1365-2133.1986.tb02831.x. PMID 3954957.
  12. ↑ Cox, NH; Mitchell, JN; Morley, WN (Dec 1986). "Lichen sclerosus et atrophicus in non-identical female twins.". The British journal of dermatology 115 (6): 743–746. doi:10.1111/j.1365-2133.1986.tb06659.x. PMID 3801314.
  13. ↑ Sherman, V; McPherson, T; Baldo, M; Salim, A; Gao, XH; Wojnarowska, F (Sep 2010). "The high rate of familial lichen sclerosus suggests a genetic contribution: an observational cohort study.". Journal of the European Academy of Dermatology and Venereology : JEADV 24 (9): 1031–1034. doi:10.1111/j.1468-3083.2010.03572.x. PMID 20202060.
  14. ↑ Meyrick Thomas, RH; Ridley, CM; McGibbon, DH; Black, MM (1988). "Lichen sclerosus et atrophicus and autoimmunity—a study of 350 women". Br J Dermatol 118 (1): 41–46. doi:10.1111/j.1365-2133.1988.tb01748.x. PMID 3342175.
  15. ↑ Drut, RM; Gomez, MA; Drut, R; Lojo, MM (1998). "Human papillomavirus is present in some cases of childhood penile lichen sclerosus: an in situ hybridization and SP-PCR study". Pediatr Dermatol 15 (2): 85–90. doi:10.1046/j.1525-1470.1998.1998015085.x. PMID 9572688.
  16. ↑ Yashar, S; Han, KF; Haley, JC (2004). "Lichen sclerosus-lichen planus overlap in a patient with hepatitis C virus infection". Br J Dermatol 150 (1): 168–169. doi:10.1111/j.1365-2133.2004.05707.x. PMID 14746647.
  17. ↑ Eisendle, K; Grabner, TG; Kutzner, H (2008). "Possible Role of Borrelia burgdorferi Sensu Lato Infection in Lichen Sclerosus". Br J Dermatol 144 (5): 591–598. doi:10.1001/archderm.144.5.591. PMID 18490585.
  18. ↑ Pass, CJ (1984). "An unusual variant of lichen sclerosus et atrophicus: delayed appearance in a surgical scar". Cutis 33 (4): 405. PMID 6723373.
  19. ↑ Milligan, A; Graham-Brown, RA; Burns, DA (1988). "Lichen sclerosus et atrophicus following sunburn". Clin Exp Dermatol 13 (1): 36–37. PMID 3208439.
  20. ↑ Yates, VM; King, CM; Dave, VK (1985). "Lichen sclerosus et atrophicus following radiation therapy". Arch Dermatol 121 (8): 1044–1047. doi:10.1001/archderm.121.8.1044. PMID 4026344.
  21. ↑ Lichen Sclerosus et Atrophicus at eMedicine
  22. 1 2 Shelley, W. B.; Shelley, E. D.; Amurao, C. V. (2006). "Treatment of lichen sclerosus with antibiotics". International Journal of Dermatology 45 (9): 1104–1106. doi:10.1111/j.1365-4632.2006.02978.x. PMID 16961523.
  23. 1 2 Chi, CC; Kirtschig, G; Baldo, M; Lewis, F; Wang, SH; Wojnarowska, F (Aug 2012). "Systematic review and meta-analysis of randomized controlled trials on topical interventions for genital lichen sclerosus.". Journal of the American Academy of Dermatology 67 (2): 305–12. doi:10.1016/j.jaad.2012.02.044. PMID 22483994.
  24. 1 2 3 4 5 "ACOG Practice Bulletin No. 93: diagnosis and management of vulvar skin disorders.". Obstet Gynecol 111 (5): 1243–53. May 2008. doi:10.1097/AOG.0b013e31817578ba. PMID 18448767.
  25. ↑ Goolamali, SK; Goolamali, SI (1997). "Lichen sclerosus". Journal of obstetrics and gynaecology 17 (1): 5–12. doi:10.1080/01443619750113997. PMID 15511755.
  26. ↑ Powell J, Wojnarowska F (2002). "Childhood vulvar lichen sclerosus: the course after puberty". J Reprod Med. 47: 706–9.
  27. ↑ Casabona, F; Priano, V; Vallerino, V; Cogliandro, A; Lavagnino, G (2010). "New surgical approach to lichen sclerosus of the vulva: The role of adipose-derived mesenchymal cells and platelet-rich plasma in tissue regeneration". Plastic and Reconstructive Surgery 126 (4): 210e–211e. doi:10.1097/PRS.0b013e3181ea9386. PMID 20885230.
  28. ↑ Li, Y; Xiao, Y; Wang, H; Li, H; Luo, X (Aug 2013). "Low-concentration topical tacrolimus for the treatment of anogenital lichen sclerosus in childhood: maintenance treatment to reduce recurrence.". Journal of pediatric and adolescent gynecology 26 (4): 239–42. doi:10.1016/j.jpag.2012.11.010. PMID 24049806.
  29. ↑ Maassen, MS; van Doorn, HC (2012). "[Topical treatment of vulvar lichen sclerosus with calcineurin inhibitors].". Nederlands tijdschrift voor geneeskunde 156 (36): A3908. PMID 22951124.
  30. ↑ National Vulvodynia Association. "Vulvodynia Fact Sheet". Vulvodynia Media Corner. National Vulvodynia Association. Retrieved 16 June 2012.
  31. ↑ Smith SD, Fischer G (2009). "Childhood onset vulvar lichen sclerosus does not resolve at puberty: a prospective case series". Pediatr Dermatol 26 (6): 725–9. doi:10.1111/j.1525-1470.2009.01022.x.
  32. ↑ Nasca, MR; Innocenzi, D; Micali, G (1999). "Penile cancer among patients with genital lichen sclerosus". J Am Acad Dermatol 41 (6): 911–914. doi:10.1016/S0190-9622(99)70245-8. PMID 10570372.
  33. ↑ Poulsen, H; Junge, J; Vyberg, M; Horn, T; Lundvall, F (2003). "Small vulvar squamous cell carcinomas and adjacent tissues. A morphologic study". APMIS 111 (9): 835–842. doi:10.1034/j.1600-0463.2003.1110901.x. PMID 14510640.
  34. ↑ Barbagli, G; Palminteri, E; Mirri, F; Guazzoni, G; Turini, D; Lazzeri, M (2006). "Penile carcinoma in patients with genital lichen sclerosus: a multicenter survey". J Urol 175 (4): 1359–1363. doi:10.1016/S0022-5347(05)00735-4. PMID 16515998.
  35. ↑ van de Nieuwenhof, HP; van der Avoort, IA; de Hullu, JA (2008). "Review of squamous premalignant vulvar lesions". Crit Rev Oncol Hematol 68 (2): 131–156. doi:10.1016/j.critrevonc.2008.02.012. PMID 18406622.
  36. ↑ Henquet, CJ (Aug 2011). "Anogenital malignancies and pre-malignancies.". Journal of the European Academy of Dermatology and Venereology : JEADV 25 (8): 885–95. doi:10.1111/j.1468-3083.2010.03969.x. PMID 21272092.
  37. ↑ Hallopeau, H (1887). "Du lichen plan et particulièrement de sa forme atrophique: lichen plan scléreux". Ann Dermatol Syphiligr (Paris) (8): 790–791.
  38. ↑ Friedrich Jr., EG (1976). "Lichen sclerosus". J Reprod Med 17 (3): 147–154. PMID 135083.

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