Lichen sclerosus (LS) is a chronic inflammatory dermatosis that results in white plaques with epidermal atrophy and scarring.

1. BY
SAAD RAHEEM ABED
Lichen Sclerosus et Atrophicus

2.  Lichen sclerosus
(LS) is a chronic
inflammatory
dermatosis that
results in white
plaques with
epidermal atrophy
and scarring.
Lichen sclerosus

3.  Lichen sclerosus has both
genital and extragenital
presentations and also
goes by the names lichen
sclerosus et
atrophicus, balanitis
xerotica obliterans (glans
penis presentation), and
kraurosis vulvae.

4. An increased
risk
of sequamous
cell carcinoma
may exist in
genital disease.
Sequamous cell carcinoma

5. Etiology and Pathogenesis
 The etiology and pathogenesis of lichen sclerosus
is unknown but may include:
● Autoimmune factors.
● Genetic factors.
● Hormonal factors.
● Infections.
● Trauma.

6. Autoimmune Factors
 Arguments supporting the hypothesis that LS is an
autoimmune disease include, on the one hand, the
greater prevalence of autoimmune diseases reported in
patients with LS and, on the other hand, the presence of
autoantibodies and a family history of immune disease.
 Recently, low-titer autoantibodies against the
extracellular matrix protein-1 (ECM-1) and collagen XVII
have been identified in 67 % of LS.
 Antibodies to basement membrane protein bp180 has
been detected in children with vulval LS lesions in 4 OF 9
lesions analyzed. All antibodies of IgG type.

7. Genetic Factors
 Genetic factors have been proposed as underlying
the development of LS based on the presence of the
disease in several family members.
 In identical twins and in nonidentical twins, various
studies have demonstrated that patients with HLA-
DQ715 are at higher risk of presenting LS.
 Interleukin 1 receptor antagonist gene
polymorphism has also been associated with the
severity of LS.

8. Hormonal Factors
 Sex hormones are considered to be an influential factor
in the development of LS.
 On the one hand, peak incidence coincides with
decreased estrogen levels, such as during premenarche
and menopause, and thus a relevant role has been
attributed to estrogens in the development of LS.
 Decreased levels of testosterone, androstenedione, and
dihydrotestosterone in patients with LS have also been
observed.
 Despite this, treatment with estrogens and testosterone
has not demonstrated clear benefit in these patients.

9. Infection
 Several microorganisms have been
associated with the appearance LS lesions:
● Borrelia burgdorferi
● Hepatitis C virus.
● Human papillomavirus.

10. Koebner Phenomenon
 In LS, as in other skin diseases, lesions are
more often found in areas that have
undergone trauma.
 Cases of LS have been reported following
sunburn and radiation therapy, and after
surgery, as occurs around vulvectomy scars.

11.  Epidemiology
 Frequency
 International
 The population rate is unknown. Male genital lichen sclerosus
is seen almost exclusively in uncircumcised or incompletely
circumcised men and boys.
 Mortality/Morbidity
 Lichen sclerosus has no associated increased mortality unless
the patient develops a malignancy in the area.
 Extragenital cases and many genital cases are asymptomatic
except for the cosmetic aspect or pruritus.
 Recalcitrant cases, especially those associated with erosion or
progressive scarring, may result in severe sexual dysfunction.

12.  The male-to-female ratio is 1:6, with female genital
cases making up the bulk of reports.
 Up to 15% of cases are in children with the majority
being vulvar presentations.

13. History
 History
 Extragenital lichen sclerosus may be asymptomatic
or it may itch, although itching is not usual.
 Vulvar lichen sclerosus usually presents with
progressive pruritus, dyspareunia, dysuria, or genital
bleeding.
 Penile lichen sclerosus usually is preceded by
pruritus but may present with sudden phimosis of
previously retractable foreskin, and urinary
obstruction can result.

14.  Skin primary
lesion
 Lichen sclerosus
usually begins as
white, polygonal
papules that
coalesce into
plaques.
Skin primary lesion

15.  Evenly spaced dells or
comedolike plugs
correspond to obliterated
appendiceal ostia.
 These may be easily
identified with
dermoscopy, keeping in
mind that other
conditions such as
chronic cutaneous lupus
may also show follicular
plugs.

16.  With time, the plugs and
dells will disappear and
leave a smooth, porcelain-
white plaque.
 Skin color is white, often
with a shiny porcelain
appearance.
 Telangiectases and
follicular plugs may be
seen.
 The size of the plaque or
plaques may vary widely
from a few millimeters
resembling lichen nitidus
to the entire upper trunk.

17.  Vulvar lichen sclerosus may progress to gradual
obliteration of the labia minora and stenosis of the
introitus.
 The most common variation occurs when the
inflammation is intense enough to cause separation
of a large area of epidermis, creating blisters or large,
occasionally hemorrhagic, bullae.
 Because this occurs more often in genital cases, it
may be confused with the trauma of sexual abuse or
other genital ulcerative disease.

18.  Given the high frequency
of genital mucosal
disease, it is surprising
that more oral cases have
not been reported.
 Those rare cases
reported are usually seen
in patients with
widespread, generalized
lichen sclerosus.

19. Clinical Features
 Genital “female lesions”.
 Genital “male lesions”.
 Perianal pyramidal protrusion.
 Extragenital including mucosal lesions.

20. Female genital LS
 The presentation of LS is usually similar in both sexes, with
the appearance of erythematous papules that coalesce
initially into erythematous plaques and then become white
and hard.
 In women, the most frequent location is usually the
anoperineal region, forming a typical figure-8 pattern
around the labia minora and anus, without affecting the
vagina or hymen.
 It usually presents with pruritus, dysuria, dyspareunia, or
pain on defecation, the most frequent symptom in girls and
a cause of constipation.
 If the inflammatory process is intense and long-lasting,
atrophy, retraction of the vulva, and synechiae of the labia
minora that alter the structure of the external genitalia may
occur.

21. Female genital LS
Lichen sclerosus in a 6-year-old girl. Typical figure-8
pattern with perineal fissure.
Lichen sclerosus in a 50-year-old woman. Atrophic
white plaques in a figure-8 pattern.

22. Male genital LS
 In men, anal involvement is rare, and the disease is
usually limited to the glans penis and prepuce. This
may lead to difficulties in retraction and pain
during erection.
 A retrospective study of 522 patients reported that
the glans penis and prepuce were affected in 57%,
the meatus in 4% and the urethra in 20%.
 Symptoms usually begin as in women, with
erythematous papules that turn white and then
progress to an atrophic band that can lead to
phimosis, paraphimosis, and urethral stenosis.

23.  Often, an hourglass,
butterfly, or figure-8
pattern involves the
perivaginal and perianal
areas, with minimal
involvement of the
perineum in between.

24.  advanced vulvar lichen
sclerosus; eroded areas
need to be carefully
examined and a biopsy
sample should be taken
to exclude coexistent
squamous cell
carcinoma.
Advanced vulvar lichen sclerosus

25. Male genital lesions
 Male genital lesions
usually are confined to
the glans penis and the
prepuce or foreskin
remnants.
 Penile shaft involvement
is much less common,
and scrotal involvement
is rare. The initial
manifestation may be a
sclerotic ring at the
prepuce edge.

26. Male genital LS
Lichen sclerosus in a 40-year-old
man. Shiny erythematous
plaques without infiltrates on the
glans penis
.

27. Perianal pyramidal protrusion
 The term infantile
perineal pyramidal
protrusion -IPPP-
defines a benign
condition clinically
characterized by a
solitary pyramidal
protrusion, pink or skin
colored, localized on the
median perineal region
in front of the anus,
mainly affecting females.

28. Perianal pyramidal protrusion
 The first reports of
perianal protrusions in
prepubertal girls date
back to 1989. Since then
about 90 cases of IPPP
have been reported.
 This exophytic lesion
that develops in the
perianal region presents
histological signs
compatible with LS.

29. Extragenital LS
 Extragenital involvement
occurs in 15% to 20% of
the patients, with
plaques that resemble
plaque morphea that are
usually asymptomatic.
 They may be located on
any part of the body
(most often on the upper
back, neck and
abdomen).

30. Mucosal LS
 The oral mucosa is rarely affected, with few cases
reported in the medical literature. It usually presents
as asymptomatic white plaques that affect the oral
mucosa and labial mucosa.

31. Complications
 Synechiae: these particularly affect the labia minora,
forming adhesions that may surround the clitoris and lead
to phimosis.
 In men, these adhesions cause phimosis and paraphimosis
of the prepuce, and may also lead to urethral stenosis.
 Infections: these result from scratching and manipulation of
the affected region.
 Epidermoid carcinoma: in women, the risk of malignancy is
4% to 6%,whereas recent studies have shown this to be
around 8% in men.
 Malignancies have not been reported in extragenital
regions.
 Sexual problems: dyspareunia, vulvodynia, and decreased
libido.
 Constipation: this is usually a complication found in
untreated children arising from discomfort on defecation.

32. Lichen Sclerosus and Squamous Cell
Carcinoma

33. Laboratory Studies
 Laboratory Studies
 Skin biopsy (punch preferred) is the primary study
to perform for diagnosis of lichen sclerosus.
 Despite the presence of autoantibodies described
in several studies, an autoimmune workup is still
not generally recommended.

34. Histologic Findings
 Histologic
Findings
 Classic lichen sclerosus
demonstrates a lichenoid
infiltrate in the dermal-
epidermal junction,
compact hyperkeratosis
with stratum corneum,
which often is thicker
than the greatly effaced
epidermis.

35.  Remarkable edema in
the papillary (upper)
dermis is replaced by a
dense, homogenous
fibrosis as the lesion
matures.
 Extensive and deeper
biopsies may show areas
more consistent with
scleroderma than classic
lichen sclerosus.

39. Differential Diagnosis
 In children, the differential diagnosis should take
into account lesions resulting from sexual abuse,
since these can present as erosions, fissures,
hematomas, bleeding, and secondary scars in the
anogenital area.
 Several articles report that in certain cases there
is an association between LS and previous sexual
abuse (Koebner phenomenon).

40. Differential Diagnosis
● Erosive vulval lichen planus or atrophic forms
● Vitiligo
● Postinflammatory hypopigmentation
● Morphea
● Postmenopausal atrophy
● Cicatricial pemphigoid
● Atrophic candidal vulvitis
● Leukoplakias
 Acrodermatitis Chronica Atrophicans

41. Treatment
 If a patient has suspected LS, a complete medical
history should be taken and any personal and
family background of immune disease thoroughly
explored (vitiligo, symptoms of diabetes, thyroid
disease symptoms, alopecia areata, or digestive
symptoms).
 Physical examination should rule out extragenital
involvement, including involvement of the oral
mucosa.
 Signs of active disease should be noted: erosions,
petekias, hemorrhages, and surface hyperkeratosis.

42.  Asymptomatic extragenital lichen sclerosus usually requires
no treatment as control of pruritus rather than resolution of
the lesion, which is a more realistic goal of therapy.
 The treatment goals are to reduce irritation, burning
sensations and pain, minimize scarring, and prevent
malignant transformation.
 Before beginning pharmacological treatment, basic hygiene
measures should be recommended: neutral soap should be
used, irritants avoided, and cotton underwear used although
this should be worn as little possible, especially at night.
 Emollients and lubricants should be used if needed and any
infections should be detected and treated.

43.  The pharmacological treatment of choice in LS is highly
potent topical corticosteroids, such as 0.05% clobetasol
propionate, in children and in adults.
 Treatment should begin with 1 or 2 applications per day
for 4 weeks, continue with 1 application every 48 hours
for another 4 weeks and, subsequently, 2 or 3
applications per week for 1 month more.
 A checkup is recommended after 3 months of treatment,
and if symptoms of activity persist, topical
corticosteroids should be maintained (2 or 3 applications
per week) or be replaced by topical 0.1% tacrolimus or
topical 1% pimecrolimus 3 times per week.

44.  Therapy with tazarotene (Tazorac) is off label for this medication
in this location and for this indication.
 Especially in genital and other occluded areas, short-contact
therapy is used, in which the gel (or cream) is initially applied for
15 min and washed off. Every 2-3 wk, the time applied may be
increased by about 15 min until either therapeutic effect or
limiting adverse effects are noted.
 If a patient is applying the medication for 3 h or more, they may
consider leaving the medication in place.
 For extragenital lichen sclerosus, this may be applied and left in
place. This may be done in conjunction with topical steroid use.
 Tazarotene has not been well studied for lichen sclerosus in
children, but application should be similar to adult usage.
 A pregnancy test is recommended before starting therapy, and the
drug is category X (contraindicated).
 Tazarotene may be irritating and is not likely to be tolerated on
open and denuded areas.

45.  Other treatments for LS have been used, but none has
proven to be more effective than topical corticosteroids
in clinical trials. Topical 2% testosterone, despite being
more effective than placebo, is not superior to
corticosteroids in the treatment of LS.
 The use of topical 0.005% calcipotriol applied daily for
1 week increasing to 2 daily applications for several
months will alleviate the pruritus.
 Other treatments, such as carbon dioxide laser therapy,
cryosurgery, photodynamic therapy and phototherapy
(ultraviolet A161 and psoralen with ultraviolet lead to
improvements in symptomatology, although the
lesions persist, and require many treatment sessions
without achieving good cosmetic results.
 In patients resistant to topical treatment, oral retinoids
can be used with good long-term results.

46.  Surgery is reserved for the majority of the complications.
Adhesions and vulval synechiae should be treated by genital
reconstruction, despite the risk of recurrence.
 In men, circumcision is the treatment of choice for lesions
that cause phimosis, and urethral dilatation in cases of
urethral stenosis.
 In all cases complicated by carcinoma, surgery is the
treatment of choice.
 All patients with LS symptoms should be treated due to the
risk of malignancy and to improve the quality of life.
 The situation is less clear in asymptomatic patients and in
children. Each case should be considered on its own merits
and the advantages and disadvantages assessed, since
corticosteroid treatment involves several risks, but these
should be balanced against the risk of developing
carcinoma.