Lichen sclerosusBMJ 2010; 340 doi: https://doi.org/10.1136/bmj.c731 (Published 15 February 2010) Cite this as: BMJ 2010;340:c731
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We read Dalziel and Shaw's article on lichen sclerosus with interest.
However, although the need for biopsy to confirm the diagnosis of lichen
sclerosus was mentioned we feel that the role of biopsy in excluding the
alternative diagnosis of malignancy needs to be emphasised.
Although vulvar cancer is still relatively uncommon, it is increasing
in incidence especially in younger women. In the UK in 2006 over 1,000
women were diagnosed with vulvar cancer, 15% of these women were under the
age of 50 years. In 1975, women under the age of 50 years made up just 6%
of new diagnoses of vulvar cancer(1).
Squamous cell carcinoma of the vulva is most commonly seen secondary
to chronic dermatoses such as lichen sclerosus; however, it may
alternatively be associated with human papillomavirus infection (2). It is
believed that the increase in the prevalence of HPV within the UK
population may account for the rising incidence of squamous cell carcinoma
of the vulva especially in younger women (3). Malignancy of either
aetiology is preceded by vulvar intraepithelial neoplasia (VIN) which
commonly presents as localised puritis or “lumps” on the vulvar (4).
However, due to its heterogeneous features VIN cannot be reliably
distinguished clinically from squamous cell carcinoma or benign vulval
Similarly, anal squamous call carcinoma is preceded by anal
intraepithelial neoplasia (AIN) for which there is no specific clinical
presentation. Areas of AIN may be scaly, raised, erythematous, pigmented
or white or show no visible change (5). Areas of AIN which are white may
be indistinguishable from lichen sclerosus by the naked eye.
Because squamous cell carcinoma, AIN and VIN lack pathognomonic
features, white perineal plaques, even in the presence of “clinical
features” of lichen sclerosus such as itch or soreness, cannot be assumed
to be benign. A biopsy should be performed for all patients not just to
confirm lichen sclerosus but more importantly to exclude malignancy.
2.Trimble C.L et al Heterogeneous etiology of squamous cell carcinoma
of the vulva. Obstetrics and Gynecology 1996. 87(1) p59-64
3.Jones R.W, Baranyai J, Stables S, Trends in squamous cell carcinoma
of the vulva: The influence of vulvar intraepithelial neoplasia Obstetrics
and Gynecology. 1997. 90(3): p. 448-452
4.MacLean A.B, Jones R.W, Scurry J, Neill S, Vulvar cancer and the
need for awareness of precursor lesions. Journal of Lower Genital Tract
Disease. 2009 13(2): p.115-117
5.Abbasakoor F, Boulos P, Anal intraepithelial neoplasia. British
Journal of Surgery 2005 92(3) p277-290
Competing interests: No competing interests
I read with curiosity the article by Dalziel and Shaw about Lichen
Sclerosus (LS). I’d like to emphasize that there’s the possibility that
the lesion may be the result of an infection. That’s why we should also
investigate about that.
There are a few microorganisms that have been related to the
appearance of the lesions of LS: Hepatitis C Virus (1), Human Papilloma
Virus (2-3-4) and Borrelia burgdorferi.
I’d expecially like to point out the link between Borrelia burgdorferi and
Studies have produced conflicting results, some reports suggesting a
positive association, others not (5-6). This has led to the hypothesis
that regional variations in B. burgdorferi may be important in dictating
the spectrum of clinical disease following infection, with LS only being
caused by certain subspecies endemic to specific geographical areas (7).
The known spectrum of skin manifestations in cutaneous Lyme disease
is continuously enlarging and can not be considered as completed. Besides
the classical manifestations of cutaneous borreliosis like erythema
(chronicum) migrans, borrelial lymphocytoma and acrodermatitis chronica
atrophicans , evidence is growing that at least in part also other skin
manifestations, especially morphea, LS and cases of cutaneous B-cell
lymphoma, are causally related to infections with Borrelia (9-10-11). In
fact borrelial infection may cause sclerotic and atrophic skin lesions
which are clinically and histopathologically indistinguishable from
morphea and LS in up to 10% of patients with ACA (10).
Studies reporting a positive association between B. burgdorferi
infection and LS have shown evidence of the organism in between 26% and
100% of cases (7-12)
B. burgdorferi was detected in patients with LS by several different
methods. Some studies used indirect immunofluorescent test to find out the
titer of antibodies (13), others detected B. burgdorferi in the lesions of
LS by PCR techniques (14-15), a recent study assessed the evidence for
borrelial infection in patients with LS by focus-floating microscopy (16).
The demonstration of the link between LS and B. burgdorferi infection
is very important for the management of LS. Current therapy centers on
topical steroids, particularly clobetasol propionate, but some patients
respond poorly to treatment. A recent study investigated the effect of
penicillin and cephalosporin therapy on patients with lichen sclerosus who
didn’t respond to potent topical corticosteroids. Most of patients showed
a rapid relief of pain, pruritus and burning, a few cleared completely
1.Ena P., Lorrai P., Pintus A., Marras V., Dessy L.A. Development of
multifocal squamous cell carcinoma in lichen sclerosus et atrophicus of
the penis associated to HCV hepatitis. Andrologia. 2004; 36: 38-40.
2.Karram M., Tabor B., Smotkin D., Wettstein F., Bhatia N., Micha J.
Detection of human papillomavirus deoxyribonucleic acid from vulvar
dystrophies and vulvar intraepithelial neoplastic lesions. Am J Obstet
Gynecol. 1988; 159:22-3
3.Neil S.M., Leibowitch M., Pelisse M.E. Lichen sclerosus, invasive
squamous cell carcinoma, and human papillomavirus. Am J Obstet Gynecol.
4.Kiene P., Mild-Langosh K., Löning T. Human papillomavirus infection
in vulvar lesions of lichen sclerosus et atrophicus. Arch Dermatol. 1991;
5.Edmonds E, Mavin S, Francis N, Ho-Yen D, Bunker C. Borrelia
burgdorferi is not associated with genital lichen sclerosus in men. Br J
Dermatol. 2009 Feb;160(2):459-60
6.Colomé-Grimmer MI, Payne DA, Tyring SK, Sánchez RL. Borrelia
burgdorferi DNA and Borrelia hermsii DNA are not associated with morphea
or lichen sclerosus et atrophicus in the southwestern United States. Acta
Derm Venereol. 1997 Jul;77(4):299-304.
7.Fujiwara H, Fujiwara K, Hashimoto K, et al. Detection of Borrelia
burgdorferi DNA (B. garinii or B. afzelii) in morphoea and lichen
sclerosus et atrophicus tissues of German and Japanese but not of US
patients. Arch Dermatol 1997;133:41–4.
8.Eisendle K, Zelger B. The expanding spectrum of cutaneous
borreliosis. G Ital Dermatol Venereol. 2009 Apr;144(2):157-71.
9.Kaya G, Berset M, Prins C, Chavaz P, Saurat JH. Chronic borreliosis
presenting with morphea- and lichen sclerosus et atrophicus-like cutaneous
lesions. a case report. Dermatology. 2001;202(4):373-5
10.Asbrink E, Brehmer-Andersson E, Hovmark A. Acrodermatitis chronica
atrophicans--a spirochetosis. Clinical and histopathological picture based
on 32 patients; course and relationship to erythema chronicum migrans
Afzelius. Am J Dermatopathol. 1986 Jun;8(3):209-19.
11.Trevisan G, Rees DH, Stinco G. Borrelia burgdorferi and localized
scleroderma. Clin Dermatol. 1994 Jul-Sep;12(3):475-9.
12.Schempp C, Bocklage H, Lange R, Kölmel HW, Orfanos CE, Gollnick H.
Further evidence for Borrelia burgdorferi infection in morphea and lichen
sclerosus et atrophicus confirmed by DNA amplification. J Invest Dermatol.
1993 May; 100(5):717-20.
13.Svecova D, Buchvald J. Borrelia burgdorferi antibodies in
scleroderma circumscripta, lichen sclerosus et atrophicus, erythema
nodosum, granuloma annulare, erythema annulare and chronic urticaria. Int
J Dermatol. 2000 Apr;39(4):278-83.
14.Ozkan S, Atabey N, Fetil E, Erkizan V, Günes AT. Evidence for
Borrelia burgdorferi in morphea and lichen sclerosus. Int J Dermatol. 2000
15.Breier F, Khanakah G, Stanek G, Kunz G, Aberer E, Schmidt B,
Tappeiner G. Isolation and polymerase chain reaction typing of Borrelia
afzelii from a skin lesion in a seronegative patient with generalized
ulcerating bullous lichen sclerosus et atrophicus. Bratisl Lek Listy.
16.Eisendle K, Grabner T, Kutzner H, Zelger B. Possible role of
Borrelia burgdorferi sensu lato infection in lichen sclerosus. Arch
Dermatol. 2008 May;144(5):662-3.
17.Shelley WB, Shelley ED, Amurao CV. Treatment of lichen sclerosus
with antibiotics. Int J Dermatol. 2006 Sep;45(9):1104-6.
Competing interests: No competing interests
We read with interest the article by Dalziel and Shaw regarding
Sclerosus (LS). We would like to point out that there is another fact that
be easily missed when visiting a patient affected by LS, i.e. the
that LS may involve also extragenital sites. Extragenital involvement may
fact be even more easily missed since these lesions are generally not
symptomatic, unlike the genital ones. Therefore it is not obvious that
would report them. Extragenital involvement appears in 10% to 15% of
patients (1) and generally on thigh, neck, trunk and breasts. Furthermore
cancerization has been described (2). Rarely oral mucosa may also be
Sometimes it may difficult to differentiate between LS and localized
scleroderma (morphea) and someone suggest that the two entities may share
a common pathologic mechanism. Antinuclear antibodies may be positive in
The authors also state that no diagnostic biochemical or immunological
investigations exist. In fact autoantibodies against extracellular matrix
protein 1 (ECM1) have been found in a big proportion of patients with LS
according to some studies (3).
The authors also correctly state that LS and children abuse can
that the latter must be always be considered. It should be noted that some
cases of LS have been linked to trauma to anogenital region. In fact a
series study concerning the aetiology of LS in children found a high rate
coexistent LS and children abuse (4).
1. Simpkin S, Oakley A. Clinical review of 202 patients with vulval
sclerosus: A possible association with psoriasis. Australas J Dermatol.
2. Sergeant A et al. Squamous cell carcinoma arising in extragenital
sclerosus. Clin Exp Dermatol. 2009 Oct;34(7):e278-9.
3. Oyama N et al. Autoantibodies to extracellular matrix protein 1 in
sclerosus. Lancet. 2003 Jul 12;362(9378):118-23.
4. Warrington SA, de San Lazaro C. Lichen sclerosus et atrophicus and
abuse. Arch Dis Child. 1996 Dec;75(6):512-6.
Competing interests: No competing interests