Lichen sclerosus
Encyclopedia
Lichen sclerosus (also known as "Lichen sclerosus et atrophicus") is an uncommon disease of unknown cause that results in white patches on the skin, which may cause scarring on and around genital skin.
Several risk factors have been proposed, including autoimmune disease
s, infection
s and genetic predisposition. There is evidence that LS can be associated with thyroid disease
.
(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. 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.
, 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).
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.
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.
l 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 and hepatitis C
are also suspected.
A link with Lyme Disease is shown by the presense of Borrelia Burgdorferi in LSA biopsy tissue.
state, hormonal influences were postulated. To date though, very little evidence has been found to support this theory.
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 there is suspicion of cancer or VIN. Histologically there's hyperkeratosis
, atrophic epidermis, sclerosis
of dermis
and lymphocyte
activity in dermis. The biopsies are also checked for signs of dysplasia
.
. These can relieve symptoms and prevent scarring.
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 cell
s in site may reduce symptoms and improve lesions. The usefulness of this treatment in males is under study.
A recent study has shown long term antibiotic treatment to be effective with all patients showing a significant response within a few weeks.
Walter B. Shelley MD, PhD, E. Dorinda Shelley MD, Cristine V. Amurao MDArticle first published online: 31 AUG 2006
DOI: 10.1111/j.1365-4632.2006.02978.x
Issue
International Journal of Dermatology
Volume 45, Issue 9, pages 1104–1106, September 2006
Lichen sclerosus is associated with a higher risk of cancer. 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. Periodic consultation is therefore necessary.
Medical pictures
Several risk factors have been proposed, including autoimmune disease
Autoimmune disease
Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. In other words, the body actually attacks its own cells. The immune system mistakes some part of the body as a pathogen and attacks it. This may be restricted to...
s, infection
Infection
An infection is the colonization of a host organism by parasite species. Infecting parasites seek to use the host's resources to reproduce, often resulting in disease...
s and genetic predisposition. There is evidence that LS can be associated with thyroid disease
Thyroid
The thyroid gland or simply, the thyroid , in vertebrate anatomy, is one of the largest endocrine glands. The thyroid gland is found in the neck, below the thyroid cartilage...
.
Synonyms and short history
Lichen sclerosus (LS) is also known as lichen sclerosus et atrophicus (LSA), balanitis xerotica obliteransBalanitis xerotica obliterans
Balanitis xerotica obliterans is a dermatological condition affecting the male genitalia. It was first described by Stuhmer in 1928, though earlier reports describe what may have been the same condition....
(BXO), Csillag's disease, White Spot Disease and kraurosis vulvae
Kraurosis vulvae
Kraurosis vulvae is a cutaneous condition characterized by atrophy and shrinkage of the skin of the vagina and vulva often accompanied by a chronic inflammatory reaction in the deeper tissues....
. 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. 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.
Signs and symptoms
Women are more commonly affected than men (10 to 1 ratio), particularly around and after menopauseMenopause
Menopause is a term used to describe the permanent cessation of the primary functions of the human ovaries: the ripening and release of ova and the release of hormones that cause both the creation of the uterine lining and the subsequent shedding of the uterine lining...
, but younger women or girls may also develop the disease. The condition most commonly occurs on the vulva
Vulva
The vulva consists of the external genital organs of the female mammal. This article deals with the vulva of the human being, although the structures are similar for other mammals....
and around the anus
Anus
The anus is an opening at the opposite end of an animal's digestive tract from the mouth. Its function is to control the expulsion of feces, unwanted semi-solid matter produced during digestion, which, depending on the type of animal, may be one or more of: matter which the animal cannot digest,...
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
Phimosis
Phimosis , from the Greek phimos ), is a condition where, in men, the foreskin cannot be fully retracted over the glans penis...
). In contrast to women, there is no perianal involvement. In men, this genital involvement has traditionally been known as balanitis xerotica obliterans
Balanitis xerotica obliterans
Balanitis xerotica obliterans is a dermatological condition affecting the male genitalia. It was first described by Stuhmer in 1928, though earlier reports describe what may have been the same condition....
(BXO).
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.
Autoimmunity
AutoimmunityAutoimmunity
Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which allows an immune response against its own cells and tissues. Any disease that results from such an aberrant immune response is termed an autoimmune disease...
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
Prevalence
In epidemiology, the prevalence of a health-related state in a statistical population is defined as the total number of cases of the risk factor in the population at a given time, or the total number of cases in the population, divided by the number of individuals in the population...
of other autoimmune diseases such as diabetes mellitus type 1
Diabetes mellitus type 1
Diabetes mellitus type 1 is a form of diabetes mellitus that results from autoimmune destruction of insulin-producing beta cells of the pancreas. The subsequent lack of insulin leads to increased blood and urine glucose...
, vitiligo
Vitiligo
Vitiligo is a condition that causes depigmentation of sections of skin. It occurs when melanocytes, the cells responsible for skin pigmentation, die or are unable to function. The cause of vitiligo is unknown, but research suggests that it may arise from autoimmune, genetic, oxidative stress,...
and thyroid disease.
Infection
Both bacteriaBacteria
Bacteria are a large domain of prokaryotic microorganisms. Typically a few micrometres in length, bacteria have a wide range of shapes, ranging from spheres to rods and spirals...
l as well as viral
Virus
A virus is a small infectious agent that can replicate only inside the living cells of organisms. Viruses infect all types of organisms, from animals and plants to bacteria and archaea...
pathogens have been implicated in the etiology of LS. A disease that is similar to LS, acrodermatitis chronica atrophicans
Acrodermatitis chronica atrophicans
Acrodermatitis chronica atrophicans is a skin rash indicative of the third or late stage of European Lyme borreliosis....
is caused by the spirochete Borrelia burgdorferi
Borrelia burgdorferi
Borrelia burgdorferi is a species of Gram negative bacteria of the spirochete class of the genus Borrelia. B. burgdorferi is predominant in North America, but also exists in Europe, and is the agent of Lyme disease....
. Viral involvement of HPV and hepatitis C
Hepatitis C
Hepatitis C is an infectious disease primarily affecting the liver, caused by the hepatitis C virus . The infection is often asymptomatic, but chronic infection can lead to scarring of the liver and ultimately to cirrhosis, which is generally apparent after many years...
are also suspected.
A link with Lyme Disease is shown by the presense of Borrelia Burgdorferi in LSA biopsy tissue.
Hormones
Since LS in females is primarily found in women with a low estrogenEstrogen
Estrogens , oestrogens , or œstrogens, are a group of compounds named for their importance in the estrous cycle of humans and other animals. They are the primary female sex hormones. Natural estrogens are steroid hormones, while some synthetic ones are non-steroidal...
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 or radiation, 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 thrushCandidiasis
Thrush redirects here. For the hoof infection see Thrush .Candidiasis or thrush is a fungal infection of any of the Candida species , of which Candida albicans is the most common...
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 there is suspicion of cancer or VIN. Histologically there's hyperkeratosis
Hyperkeratosis
Hyperkeratosis is thickening of the stratum corneum, often associated with a qualitative abnormality of the keratin, and also usually accompanied by an increase also in the granular layer...
, atrophic epidermis, sclerosis
Sclerosis (medicine)
In medicine, sclerosis refers to the stiffening of a structure, usually caused by a replacement of the normal organ-specific tissue with connective tissue.Types include:...
of dermis
Dermis
The dermis is a layer of skin between the epidermis and subcutaneous tissues, and is composed of two layers, the papillary and reticular dermis...
and lymphocyte
Lymphocyte
A lymphocyte is a type of white blood cell in the vertebrate immune system.Under the microscope, lymphocytes can be divided into large lymphocytes and small lymphocytes. Large granular lymphocytes include natural killer cells...
activity in dermis. The biopsies are also checked for signs of dysplasia
Dysplasia
Dysplasia , is a term used in pathology to refer to an abnormality of development. This generally consists of an expansion of immature cells, with a corresponding decrease in the number and location of mature cells. Dysplasia is often indicative of an early neoplastic process...
.
Treatment
LS is usually treated with potent topical steroids, like Clobetasol propionateClobetasol propionate
Clobetasol propionate is a corticosteroid used to treat various skin disorders including eczema and psoriasis. It is also highly effective for contact dermatitis caused by exposure to poison ivy/oak. Clobetasol belongs to US Class I of the corticosteroids, making it one of the most potent...
. These can relieve symptoms and prevent scarring.
Circumcision
Circumcision
Male circumcision is the surgical removal of some or all of the foreskin from the penis. The word "circumcision" comes from Latin and ....
may be recommended in males to correct phimosis
Phimosis
Phimosis , from the Greek phimos ), is a condition where, in men, the foreskin cannot be fully retracted over the glans penis...
. 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
Platelet-rich plasma
Platelet-rich plasma is blood plasma that has been enriched with platelets. As a concentrated source of autologous platelets, PRP contains several different growth factors and other cytokines that stimulate healing of bone and soft tissue.-Components:The efficacy of certain growth factors in...
) and stem cell
Stem cell
This article is about the cell type. For the medical therapy, see Stem Cell TreatmentsStem cells are biological cells found in all multicellular organisms, that can divide and differentiate into diverse specialized cell types and can self-renew to produce more stem cells...
s in site may reduce symptoms and improve lesions. The usefulness of this treatment in males is under study.
A recent study has shown long term antibiotic treatment to be effective with all patients showing a significant response within a few weeks.
Walter B. Shelley MD, PhD, E. Dorinda Shelley MD, Cristine V. Amurao MDArticle first published online: 31 AUG 2006
DOI: 10.1111/j.1365-4632.2006.02978.x
Issue
International Journal of Dermatology
Volume 45, Issue 9, pages 1104–1106, September 2006
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, particularly in young girls.Lichen sclerosus is associated with a higher risk of cancer. 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. Periodic consultation is therefore necessary.
See also
- Lichen planusLichen planusLichen planus is a chronic mucocutaneous disease that affects the skin, tongue, and oral mucosa. The disease presents itself in the form of papules, lesions, or rashes. Lichen planus does not involve lichens, the fungus/algae symbionts that often grow on tree trunks; the name refers to the dry and...
- Balanitis xerotica obliteransBalanitis xerotica obliteransBalanitis xerotica obliterans is a dermatological condition affecting the male genitalia. It was first described by Stuhmer in 1928, though earlier reports describe what may have been the same condition....
- List of cutaneous conditions
External links
- Lichen sclerosus
- OMNI
- NIAMS - Questions and Answers About Lichen Sclerosus
- NIAMS - Fast Facts About Lichen Sclerosus
- vulvarhealth.org
- dermnetnz.org
- mayoclinic.com
- better medicine
- Medscape Reference Author: Jeffrey Meffert, MD; Chief Editor: Dirk M Elston, MD
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