Lichen sclerosus

Lichen sclerosus
Lichen sclerosus
Classification and external resources

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

Lichen sclerosus (LS) (also known as "Lichen sclerosus et atrophicus"[1]:227) is an uncommon disease of unknown cause that results in white patches on the skin, which may cause scarring on and around genital skin.[2]

Several risk factors have been proposed, including autoimmune diseases, infections and genetic predisposition.[3][4] There is evidence that LS can be associated with thyroid disease.[5]

Contents

Synonyms and short history

Lichen sclerosus (LS) is also known as lichen sclerosus et atrophicus (LSA), balanitis xerotica obliterans (BXO), Csillag's disease, 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.

Lichen sclerosus et atrophicus was first described in 1887 by Dr. Hallopeau.[6] 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. Since then this is the official medical name for this disease.[7]

Signs and symptoms

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).[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]

Pathophysiology

Although it is not clear what causes LS, four theories have been postulated.

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.[10]

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[11] and hepatitis C[12] are also suspected.

Hormones

Since LS in females is primarily found in women with a low estrogen state, 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[13] or radiation,[14][15] 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.

It's rare for a biopsy of the affected skin to be taken, especially so in children. A biopsy is only performed if suspecting of cancer or VIN. Histologically there's hyperkeratosis, atrophic epidermis, sclerosis of dermis and lymphocyte activity in dermis.[16] The biopsies are also checked for signs of dysplasia.

Treatment

LS is usually treated with potent topical steroids, like Clobetasol propionate[17]. These can relieve symptoms and prevent scarring.[18]

Circumcision may be recommended in males to correct phimosis. 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. [19]

Psychology

Distress due to the discomfort LS causes is normal. So are concerns with self-esteem and sex. Counseling can help.

Prognosis

The disease can last for a considerably long time. Occasionally, "spontaneous cure" may ensue[20], particularly in young girls.

Lichen sclerosus is associated with a higher risk of cancer.[21][22][23] 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.[24] Periodic consultation is therefore necessary.

See also

References

  1. ^ James, William D.; Berger, Timothy G.; et al. (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. ^ 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. 
  4. ^ 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. 
  5. ^ Birenbaum, DL; Young, RC (2007). "High prevalence of thyroid disease in patients with lichen sclerosus". J Reprod Med 52 (1): 28–30. PMID 17286064. 
  6. ^ Hallopeau, H (1887). "Du lichen plan et particulièrement de sa forme atrophique: lichen plan scléreux". Ann Dermatol Syphiligr (Paris) (8): 790–791. 
  7. ^ Friedrich Jr., EG (1976). "Lichen sclerosus". J Reprod Med 17 (3): 147–154. PMID 135083. 
  8. ^ Balanitis Xerotica Obliterans at eMedicine
  9. ^ Laymon, CW (1951). "Lichen sclerosus et atrophicus and related disorders". MA Arch Derm Syphilol 64 (5): 620–627. PMID 14867888. 
  10. ^ Meyrick Thomas, RH; Ridley, CM; McGibbon, DH; Black, MM (1988). "Lichen sclerosus et atrophicus and autoimmunity—a study of 350 women". Br J Dermatol 188 (1): 41–46. PMID 3342175. 
  11. ^ 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. 
  12. ^ 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. 
  13. ^ Pass, CJ (1984). "An unusual variant of lichen sclerosus et atrophicus: delayed appearance in a surgical scar". Cutis 33 (4): 405. PMID 6723373. 
  14. ^ Milligan, A; Graham-Brown, RA; Burns, DA (1988). "Lichen sclerosus et atrophicus following sunburn". Clin Exp Dermatol 13 (1): 36–37. PMID 3208439. 
  15. ^ 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. 
  16. ^ Lichen Sclerosus et Atrophicus at eMedicine
  17. ^ http://en.wikipedia.org/wiki/Clobetasol_propionate
  18. ^ Goolamali, SK; Goolamali, SI (1997). "Lichen sclerosus". Journal of obstetrics and gynaecology 17 (1): 5–12. doi:10.1080/01443619750113997. PMID 15511755. 
  19. ^ http://www.ncbi.nlm.nih.gov/pubmed/20885230
  20. ^ 6.Smith SD, Fischer G. Childhood onset vulvar lichen sclerosus does not resolve at puberty: a prospective case series. Pediatr Dermatol. Nov-Dec 2009;26(6):725-9.
  21. ^ 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. 
  22. ^ Poulsen, H; Junge, J; Vyberg, M; Horn, T; Lundvall, F (2003). "Small vulvar squamous cell carcinomas and adjacent tissues. A morphologic study". APMIS 11 (9): 835–842. PMID 14510640. 
  23. ^ 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. 
  24. ^ 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. 

External links

Medical pictures

v · d · eCutaneous keratosis, ulcer, atrophy, and necrobiosis (L82–L94, 700–701.5)
Epidermal thickening

keratoderma: Keratoderma climactericum • Paraneoplastic keratoderma (Acrokeratosis paraneoplastica of Bazex )  • Aquagenic keratoderma · Drug-induced keratoderma  · Paraneoplastic keratoderma • psoriasis (Keratoderma blennorrhagica)

keratosis: Seborrheic keratosis (Clonal seborrheic keratosis · Common seborrheic keratosis · Irritated seborrheic keratosis · Seborrheic keratosis with squamous atypia · Reticulated seborrheic keratosis · Dermatosis papulosa nigra) • Keratosis punctata of the palmar creases

other hyperkeratosis: Acanthosis nigricans (Confluent and reticulated papillomatosis) · Callus  • Ichthyosis acquisita · Arsenical keratosis · Chronic scar keratosis · Hyperkeratosis lenticularis perstans · Hydrocarbon keratosis · Hyperkeratosis of the nipple and areola · Inverted follicular keratosis · Lichenoid keratosis · Multiple minute digitate hyperkeratosis · PUVA keratosis · Reactional keratosis · Stucco keratosis · Thermal keratosis · Viral keratosis · Warty dyskeratoma · Waxy keratosis of childhood

other hypertrophy: Keloid · Hypertrophic scar • Cutis verticis gyrata ·
Necrobiosis/granuloma
Granuloma annulare (Perforating, Generalized, Subcutaneous, Granuloma annulare in HIV disease, Localized granuloma annulare, Patch-type granuloma annulare) · Necrobiosis lipoidica · Annular elastolytic giant cell granuloma · Granuloma multiforme · Necrobiotic xanthogranuloma · Palisaded neutrophilic and granulomatous dermatitis · Rheumatoid nodulosis · Interstitial granulomatous dermatitis/Interstitial granulomatous drug reaction
Foreign body granuloma
Beryllium granuloma • Mercury granuloma • Silica granuloma • Silicone granuloma • Zirconium granuloma • Soot tattoo • Tattoo • Carbon stain
Other/ungrouped
eosinophilic dermatosis (Granuloma faciale)
Dermis/
localized CTD
chronic: Discoid · Panniculitis
subacute: Neonatal
ungrouped: Chilblain · Lupus erythematosus–lichen planus overlap syndrome · Tumid · Verrucous · Rowell's syndrome
Localized scleroderma (Localized morphea, Morphea–lichen sclerosus et atrophicus overlap, Generalized morphea, Atrophoderma of Pasini and Pierini, Pansclerotic morphea, Morphea profunda, Linear scleroderma)
Atrophic/
atrophoderma
Lichen sclerosus · Anetoderma (Schweninger–Buzzi anetoderma, Jadassohn–Pellizzari anetoderma) · Atrophoderma of Pasini and Pierini · Acrodermatitis chronica atrophicans · Semicircular lipoatrophy · Follicular atrophoderma · Linear atrophoderma of Moulin
Kyrle disease · Reactive perforating collagenosis · Elastosis perforans serpiginosa · Perforating folliculitis · Acquired perforating dermatosis
Other
Calcinosis cutis · Sclerodactyly · Poikiloderma vasculare atrophicans · Ainhum/Pseudo-ainhum

M: INT, SF, LCT

anat/phys/devp

noco(i/b/d/q/u/r/p/m/k/v/f)/cong/tumr(n/e/d), sysi/epon

proc, drug (D2/3/4/5/8/11)

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