28th September 17:13
scleroderma connection to Lyme disease (scleroderma balanitis urologic syphilis lichen planus)
addition to the research on the relation of Borrelia burgdorferi to
scleroderma are many reports linking LS to this spirochete. ACA, once
thought a form of scleroderma, and at times coexistent with LS,157,256 is
now well established as a borrelial disease.234,288 Researchers have found
spirochetes in the papillary dermis of as many as 48% of patients with LS,
especially in early cases.289 Six of 13 patients with predominantly genital
LS has spirochetes revealed by immunoperoxidase methods, inclduing a
possible organism in a focus of basilar degeneration........"
JOURNAL OF THE AMERICAN JOURNAL OF DERMATOLOGY, Volume 32, Number 3: Pages
Jeffrey J. Meffert, Brian M. Davis, and Ronald E. Grimwood.
"................In accordance with this standardization in nomenclature, the
term lichen sclerosus will be used unless it is unclear that all data and
opinions in some older reports refer exclusively to LS and not a blend of LS
and other disease entities (often the case in discussions of "leukoplakia"
The controversy about the relation of LS to lichen planus (LP) and
scleroderma began early and continues to this day. Hallopeau believed that LS
is a form of LP because of occasional oral findings.17Others counter with the
histologic features of oral lesions so typical of LS that the existence of
"oral LS" is recognized18-25even as the sole manifestation.26Until recently,
some authors still argued for a close relation of LS with LP, proposing that
if enough sections were cut in a biopsy specimen, typical LP would be
found.27Others, while accepting LS as a distinct entity, suggest that
Hallopeau's original cases were really atrophic LP.17Although most recent
reports do not link LS and LP except as coincidental findings, the
association of LS and morphea (localized scleroderma) remains a topic of
research and debate and will be discussed in detail later.
Divisive to the establishment of consistent principles of diagnosis and
treatment was the parallel work on the nature and therapy of different
medical disciplines. The gynecologic literature predates Hallopeau with
Breisky's description of "kraurosis vulvae" in 1885.13The urologic literature
dates back to Stühmer's description14of balanitis xerotica obliterans in
1928. Ormsby and Mitchell28first connected LS with kraurosis vulvae in 1920,
and Freeman and Laymon,29,30made the same association with balanitis xerotica
obliterans in 1941. Table I chronicles key events leading to our current
understanding of LS....................."
"..............Lichen sclerosus, usually appearing in the dermatologic
literature under the names of lichen sclerosus et atrophicus, balanitis
xerotica obliterans, and kraurosis vulvae, is an inflammatory disease with a
multifactorial origin. A past association of lichen sclerosus and genital
squamous cell carcinoma is not as close as once thought. Once considered
primarily a surgical problem, especially when the genitals are involved,
lichen sclerosus will respond to a variety of systemic and topical therapies.
Lichen sclerosus (LS), usually reported in the dermatologic literature as
lichen sclerosus et atrophicus, is an inflammatory disease of unknown cause
and incompletely characterized pathogenesis. Beginning with the initial case
reports at the turn of the century, there has been much confusion in the
literature because of the plethora of terms used to describe
"..............Year Event Key reference no.(s)
1875 Weir's report of possible vulvar and/or oral LS as "ichthyosis" 2
1885 Breisky describes kraurosis vulvae 13
1887 Hallopeau describes series of extragenital LS (and one possible vulvar
case) 1, 3-5
1892 Darier formally describes classic histopathology of LS 6, 7
1900-present Concept that scleroderma and LS are closely related 72, 73,
222, 227, 231, 290, 358
1901 Pediatric LS described 9, 53, 95, 96, 101, 103
1913-present Concept that scleroderma is not closely related to LS 45, 145,
148, 158, 238, 240, 281
1920 Taussig establishes vulvectomy as treatment of choice for kraurosis
vulvae, a premalignant condition 93, 183-185; also 79, 186, 191, 196
1927 Kyrle defines LS ("white spot disease") as entity sui generis 359
1928 Stühmer describes balanitis xerotica obliterans as postcir***cision
1936 Retinoids (vitamin A) used in LS 47, 195, 341, 349, 352, 353
1945 Testosterone used in genital LS 87, 92, 160, 254, 337, 338, 342
1961 Modern use of corticosteroids 21, 102, 165, 254, 337, 338, 342
1966 Jeffcoate presents argument against vulvectomy for simple LS 119; also
51, 198, 209
1971 Progesterone used in LS 254, 338, 344, 345
1971 Wallace defines clinical factors and epidemiology of LS for all later
1976 Friedrich defines LS as a dystrophic, not atrophic condition; "et
atrophicus" dropped 15
1976 International Society for Study of Vulvar Disease classification system.
"Kraurosis" and "leukoplakia" no longer to be used 16, 181, 182
1980 Fluourinated and superpotent steroids used in LS 54, 56, 96, 101-103,
1981 Studies into HLA serotypes and LS 44, 217, 273, 275-77, 279
1984 Etretinate and acetretin used in LS 25, 149, 343, 354-357
1987 LS linked with Borrelia infection 232, 233, 256, 289, 290
".................LS in children is histologically identical to that in
adults.55 However, it represents a special subset of LS for several reasons.
Ten percent to 15% of LS cases occur in children, the majority of which
involve the female genitalia.52 Again, the incidence is uncertain because of
delay or failure to diagnose LS, masking of LS by secondary changes,95 a
tendency for spontaneous involution, and a reluctance to seek medical care
for what may be perceived as child ***ual abuse.96 Because of the traumatic
appearance of hemorrhagic and bullous LS, it is discussed in the pediatric
literature as an entity often confused with ***ual abuse.21, 97-103 However,
it cannot be assumed that the diagnosis of LS excludes ***ual abuse. In a
7-year-old girl initial improvement with treatment was followed by dramatic
worsening and subsequent confirmation of ***ual
"............A study of risk factors for LS revealed that nonsmokers were
significantly more likely to have LS than smokers. The authors conjecture
that this is caused by the androgen-elevating effect of cigarette
"............... Careful attention to hygiene is recommended, and efforts
to treat coexistent ******l infections102,321 or balanitis are essential.
Because of the possible role of spirochetes in some cases of LS and morphea,
many recommend empirical treatment with antispirochetal antibiotics such as
penicillin or doxycycline.184,232,233,291 The utility of this therapy in LS
remains to be defined.................."
"...............CONCLUSIONS AND UNANSWERED QUESTIONS
The true incidence of LS is unknown. Significant underreporting is
likely for many reasons. These include faliure to recognize the condition,
misdiagnosis as a related or unrelated disease, frequently asymptomatic
patients, and reluctance to seek care because of embarassment or fear of
legal complications (pediatric cases). Accurate epidemiology of LS is further
hampered by fragmentation of case in various specialties (dermatology,
gynecology, urology, pediatrics, family practice), many using different
terminology for the same entity.
LS is an inflammatory condition caused by an incompletely defined
response to a variety of mechanical and antigenic stimulu. These stimuli
include, among others, trauma, spirochetal infection, and autoimmune diseases.
Further definition of the role of spirochetal diseases in LS is pivotal
in making treatment recommendations. If this is a major cause of LS, the mode
of transmission should be elucidated. Is the transmission via an insect
vector (Lyme disease), ***ually (venereal syphilis), or through casual
person-to-person contact (endemic syphilis and pinta)?
LS and scleroderma, although clinically and histologically distinct
entities that may coexist, share many pathogenic mechanisms. Perhaps they
represent expressions of the same antigenic insult with LS being expressed in
the papillary dermis and scleroderma in the reticular dermis and deeper.
Differences in vascular adhesion molecuels, elastic network, and relative
amounts of type I and III collagen may explain the differences between the
two conditions. An unidentified genetic or environmental factor may determine
which disease the patient acquires.
True LS is not itself a premalignant condition, although other white
lesions that stimulate it, such as hyperplastic dystrophy, may be. LS should
not be treated with aggressive surgical modalities. Prior radiation therapy
may have artificially increased historical rates of "malignant
transformation." The contribution of human papillomavirus to current sporadic
cases of LS-associated genital squamous cell carcinoma is unknown.
Urologists, in contrast to gynecologists, continue to view LS as
primarily a surgical problem. Cir***cision is still the most widely used
primary therapy for genital LS in uncir***cised men. Reports of medical
therapy are anecdotal and generally limited to persistent or recurrent
lesions in patients already cir***cised.
Medical therapies include retinoids and topical corticosteroids
(including superpotent agents), testosterone, and progesterone. Other
systemic and topical treatments have been used with variable success. Genital
LS in children presents some special treatment