LICHEN SCLEROSIS
Written by: Leigh Kochan, N.D. and Tori Hudson, N.D.
Lichen Sclerosis (LS), a chronic, progressive condition, is a poorly
recognized chronic inflammatory skin disorder mainly affecting
the vulvar and perianal area. The condition is relatively rare
and affects less than 1% of adult women, and its prevalence is
highest among postmenopausal caucasian women. However, it can occur
in any age group, including young girls prior to menarche. The
most common presenting symptoms and complaint of the patient with
lichen sclerosis is vulvar itching, sometimes rather intense. Early
in the disease, small, subtle white areas may appear. As time goes
on, the spots develop into bigger patches, and the skin surface
becomes thin and wrinkled. Linear fissures can also occur due to
the thinning of the vulvar tissue. Some adjacent areas may be red
and irritated. Occasionally, the itching is also accompanied by
vulvar pain, fragility of the tissue where the skin tears easily
and subsequent bleeding, along with dyspareunia. More severe cases
of LS produce scarring that may cause the vulva to shrink and the
opening of the vagina to narrow. Very mild LS of the genital area
may cause just the itching, but many cases have no symptoms at
all and is only recognized upon physical exam. Less common, LS
may cause extreme itching that interferes with sleep and daily
activities. Urination may burn and cause pain and bleeding can
occur with intercourse. An overview of common symptoms follow:
•
chronic itching and soreness of the vulvar area
•
inflammation and fissures of the vulva
•
increased susceptibility to infection
•
patches of white discoloration
•
atrophy of the vulva, causing change in shape and size of the area,
sometimes causing urination difficulties and sexual problems
•
narrowing of the vaginal opening
•
fusion of the labia, clitoral hood and urethra
•
there is a small risk, estimated at between 2% and 5%, of cancer
of the vulva in adult women
The exact cause of LS is not yet understood, but a few possible causes
are under consideration. One potential connection is through an autoimmune
process where one’s own antibodies attack the tissue causing
damage. Other triggers of LS include physical trauma, spirochete
and/or humanpapilloma virus infection and hormonal changes. The hormonal
connection is supported by the fact that the largest population of
LS sufferers are post-menopausal women and that remission may occur
at puberty and with pregnancy. In addition, the Koebner phenomenon
may be related as LS occurs in skin already scarred or damaged, so
trauma, injury, and sexual abuse may be possible triggers of symptoms
in genetically predisposed individuals.
The diagnosis of LS is often elusive and can be a long, difficult
process and patients are sometimes misdiagnosed and mistreated. Definitive
diagnosis of LS is made with a punch biopsy of the local tissue.
Correct diagnosis is essential to receive proper treatment. Lichen
Sclerosis does not cause cancer, although a woman’s risk of
developing invasive squamous cell carcinoma secondary to vulvar LS
is extremely low (2% to 5% over years time). Skin that is scarred
by untreated LS may be more likely to develop cancer and, therefore,
frequent follow up is required.
LS of the vulva should be treated, regardless of the presence or
severity of symptoms, as resulting scarring and narrowing is irreversible
and may interfere with urination and/or sexual intercourse. There
is also a very small chance that cancer may develop without appropriate
treatment. The treatment of choice is prescription topical corticosteroid
(betamethasone dipropionate, clobetasol propionate, diflorasone diacetate,
halobetasol propionate) which can stop itching within a few days,
restoring the skin's normal texture and strength within a couple
months. However, treatment does not reverse the scarring that may
have already occurred. Topical steroids may be decreased to a couple
times per week, although use must continue indefinitely, to keep
LS in remission. In the past, testosterone ointment or cream was
used, but this has not shown to be effective. Topical progesterone
may be beneficial in some patients. Other factors, such as low estrogen
levels that cause vaginal dryness and soreness, a skin infection,
or irritation or allergy to medication, may interfere with symptom
resolution. Retinoids, or vitamin A-like medications, may be helpful
for patients who cannot tolerate or are not helped by topical corticosteroids.
Treatment with oral calcitriol (1-25 dihydroxyvitamin D3) has been
suggested as it has been effective in similar conditions. The positive
effect could be due to immunomodulatory effects of calcitriol and
in one case report, the skin extensibility increased and the lesions
improved. The improvement persisted after discontinuation of therapy
during a follow-up period of one year). Some studies suggest that
UVA1, a phototherapy, may prove beneficial for LS. Although quite
long remissions can be experienced, the symptoms often return. Another
study implicates a decrease in glycosaminoglycans; essential building
blocks of tissue, suggesting that supplementation with N-acetyl glucosamine
may be beneficial. Surgery may be useful for the damage caused by
scarring, but only after LS is controlled with medication. In some
cases, symptoms can still persist with no apparent remedy and creativity
and flexibility in thinking and treatment options is warranted.
Self-care may help to decrease symptoms. Many products generally
used for washing, bathing and showering may irritate and dry the
already sensitive skin. It is better to avoid soaps, bubble baths,
talc and anything with artificial colors and/or scents. Natural emollients
should be used as often as necessary. The use of ointments is recommended
as they moisturize, last longer and protect sensitive areas from
urine which may irritate the skin. Cream-based, alcohol-free soaps
may also be less irritating. The emollient can be put on regularly
throughout the day if the area becomes sore or itchy but should not
be used at the same time as other treatment cream/ointments spaced
by 1/2 - 1 hour in between so as not to minimize the treatment’s
effectiveness. In addition, cotton underwear is best and panty liners
may protect underwear from creams/ointments which may stain.
REFERENCES
Bracco GL, Carli P, Sonni L, Maestrini G, De Marco A, Taddei
GL, Cattaneo A. Clinical and histologic effects of topical
treatments of vulval lichen sclerosis. A critical evaluation.
J Reprod Med. 1993 Jan;38(1):37-40.
Dalziel KL, Wojnarowska F. Long-term control of vulval lichen
sclerosis after treatment with a potent topical steroid cream.
J Reprod Med. 1993 Jan;38(1):25-7.
Dawe RS. Br J Dermatol. Ultraviolet A1 phototherapy 2003 Apr;148(4):626-37.
Lichen sclerosis information center http://members.tripod.com/~shanmd/lichen.html
National Institute of Arthritis and Musculosketal and Skin Diseases
http://www.niams.nih.gov/hi/topics/lichen/lichen.htm
Powell JJ, Wojnarowska F. Lichen sclerosis. Lancet. 1999 May
22; 353(9166): 1777-83.
Ronger S, Viallard AM, Meunier-Mure F, Chouvet B, Balme B, Thomas
L. J Drugs Dermatol. Oral calcitriol: a new therapeutic agent
in cutaneous lichen sclerosis.2003 Jan;2(1):23-8.
Serrano G, Millan F, Fortea JM, Grau M, Aliaga A.Topical progesterone
as treatment of choice in genital lichen sclerosis et atrophicus
in children. Pediatr Dermatol. 1993 Jun;10(2):201.
Yoshima H, Shinkai H, Sano S, Homma M.Acidic glycosaminoglycans
in skin and urine of a patient with wide-spreaded lichen sclerosis
et atrophicus. Arch Dermatol Res. 1978;263(1):59-66.
