| 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 termed, incorrectly, "Lichen sclerosus 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 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
- 1 Synonyms and short history
- 2 Signs and symptoms
- 3 Pathophysiology
- 3.1 Autoimmunity
- 3.2 Infection
- 3.3 Hormones
- 3.4 Local skin changes
- 4 Diagnosis
- 5 Treatment
- 6 Psychology
- 7 Prognosis
- 8 See also
- 9 References
- 10 External links
Synonyms and short history[edit]
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. Sometimes, LS can be found on pediatric patients and men from all ages. Children being diagnosed with Lichen Scleroses is extremely rare. 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.[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.[7]
Signs and symptoms[edit]
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[edit]
Although it is not clear what causes LS, four theories have been postulated. Lichen Sclerosus is not contagious; it cannot be caught from another person.[10]
Autoimmunity[edit]
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.[11]
Infection[edit]
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[12] and hepatitis C[13] are also suspected.
A link with Lyme Disease is shown by the presence of Borrelia burgdorferi in LSA biopsy tissue.[14]
Hormones[edit]
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[edit]
Some findings suggest that LS can be initiated through scarring[15] or radiation,[16][17] although these findings were sporadic and very uncommon.
Diagnosis[edit]
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.[18] The biopsies are also checked for signs of dysplasia.[19]
Treatment[edit]
LS is usually treated with potent topical steroids, like Clobetasol propionate. These can relieve symptoms and prevent scarring.[20]
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.[21]
A recent study has shown long-term antibiotic treatment to be effective with all patients, showing a significant response within a few weeks.[19]
Psychology[edit]
Distress due to the discomfort and pain of Lichen Sclerosus is normal, as are concerns with self-esteem and sex. Counseling can help.
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.[22] Depression, anxiety, and even anger are all normal responses to the ongoing pain LS patients suffer from.
Prognosis[edit]
The disease can last for a considerably long time. Occasionally, "spontaneous cure" may ensue,[23] particularly in young girls.
Lichen sclerosus is associated with a higher risk of cancer.[24][25][26] 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.[27] Periodic consultation is therefore necessary.
See also[edit]
- Lichen planus
- Balanitis xerotica obliterans
- List of cutaneous conditions
- List of cutaneous conditions associated with increased risk of nonmelanoma skin cancer
- List of human leukocyte antigen alleles associated with cutaneous conditions
References[edit]
- ^ James, William D.; Berger, Timothy G. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ Birenbaum, DL; Young, RC (2007). "High prevalence of thyroid disease in patients with lichen sclerosus". J Reprod Med 52 (1): 28–30. PMID 17286064.
- ^ Hallopeau, H (1887). "Du lichen plan et particulièrement de sa forme atrophique: lichen plan scléreux". Ann Dermatol Syphiligr (Paris) (8): 790–791.
- ^ Friedrich Jr., EG (1976). "Lichen sclerosus". J Reprod Med 17 (3): 147–154. PMID 135083.
- ^ Balanitis Xerotica Obliterans at eMedicine
- ^ 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.
- ^ National Institute of Health. "Fast Facts About Lichen Sclerosus". Lichen Sclerosus. National Institute of Arthritis and Musculoskeletal and Skin Diseases. Retrieved 16 June 2012.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ Pass, CJ (1984). "An unusual variant of lichen sclerosus et atrophicus: delayed appearance in a surgical scar". Cutis 33 (4): 405. PMID 6723373.
- ^ Milligan, A; Graham-Brown, RA; Burns, DA (1988). "Lichen sclerosus et atrophicus following sunburn". Clin Exp Dermatol 13 (1): 36–37. PMID 3208439.
- ^ 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.
- ^ Lichen Sclerosus et Atrophicus at eMedicine
- ^ a b 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. edit
- ^ Goolamali, SK; Goolamali, SI (1997). "Lichen sclerosus". Journal of obstetrics and gynaecology 17 (1): 5–12. doi:10.1080/01443619750113997. PMID 15511755.
- ^ 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.
- ^ National Vulvodynia Association. "Vulvodynia Fact Sheet". Vulvodynia Media Corner. National Vulvodynia Association. Retrieved 16 June 2012.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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.
- ^ 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[edit]
- Lichen sclerosus
- OMNI
- NIAMS - Questions and Answers About Lichen Sclerosus
- NIAMS - Fast Facts About Lichen Sclerosus
- dermnetnz.org
- mayoclinic.com
- better medicine
- Medscape Reference Author: Jeffrey Meffert, MD; Chief Editor: Dirk M Elston, MD
Support Groups
- [1] WLSS - Worldwide Lichen Sclerosus Support
- [2] LSRS - Lichen Sclerosus Resources and Support (and research)
- [3] Lichen Sclerosus Yahoo! Group
Medical Pictures
- http://www.dermlectures.com/LecturesWMV.cfm?lectureID=88
- http://dermis.multimedica.de/dermisroot/de/34088/diagnose.htm
- http://dermnetnz.org/immune/ls-imgs.html
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Cutaneous keratosis, ulcer, atrophy, and necrobiosis (L82–L94, 700–701.5)
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| Epidermal thickening |
- keratoderma: Keratoderma climactericum
- Paraneoplastic keratoderma
- Acrokeratosis paraneoplastica of Bazex
- Aquagenic keratoderma
- Drug-induced keratoderma
- Paraneoplastic keratoderma
- psoriasis
- Keratoderma blennorrhagicum
- 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
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|
| Necrobiosis/granuloma |
| Necrobiotic/palisading |
- 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
|
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| Foreign body granuloma |
- Beryllium granuloma
- Mercury granuloma
- Silica granuloma
- Silicone granuloma
- Zirconium granuloma
- Soot tattoo
- Tattoo
- Carbon stain
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| Other/ungrouped |
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|
|
Dermis/
localized CTD |
Cutaneous lupus
erythematosus |
- chronic: Discoid
- Panniculitis
- ungrouped: Chilblain
- Lupus erythematosus–lichen planus overlap syndrome
- Tumid
- Verrucous
- Rowell's syndrome
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|
Scleroderma/
Morphea |
- 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
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| Perforating |
- Kyrle disease
- Reactive perforating collagenosis
- Elastosis perforans serpiginosa
- Perforating folliculitis
- Acquired perforating dermatosis
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| Skin ulcer |
|
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| Other |
- Calcinosis cutis
- Sclerodactyly
- Poikiloderma vasculare atrophicans
- Ainhum/Pseudo-ainhum
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noco (i/b/d/q/u/r/p/m/k/v/f)/cong/tumr (n/e/d), sysi/epon
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proc, drug (D2/3/4/5/8/11)
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