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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 24  |  Issue : 3  |  Page : 129-131

A case of well-differentiated squamous cell carcinoma in vulval lichen sclerosus


1 Department of Dermatology, Base Hospital Delhi Cantt and Army College of Medical Sciences, New Delhi, India
2 Department of Laboratory Sciences and Molecular Medicine, Army Hospital Research and Referral, New Delhi, India

Date of Submission28-Aug-2021
Date of Decision18-Nov-2021
Date of Acceptance29-Nov-2021
Date of Web Publication01-Apr-2022

Correspondence Address:
(Dr) Pankaj Das
Department of Dermatology, Base Hospital Delhi Cantt and Army College of Medical Sciences, New Delhi - 110 010
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jmms.jmms_112_21

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  Abstract 


Lichen sclerosus (LS) et atrophicus is a known premalignant condition affecting both sexes. Squamous cell carcinoma is the most common malignancy seen in LS et atrophicus. We report a case of 42-year-old female suffering from vulval LS et atrophicus for 12 years who developed squamous cell carcinoma. It is imperative to counsel the patients with LS regarding the malignant potential and actively monitor them for better outcome in terms of survival as well as quality of life.

Keywords: Balanitis xerotica obliterans, kraurosis vulvae, lichen sclerosus, lichen sclerosus et atrophicus, squamous cell carcinoma


How to cite this article:
Das P, Arora S, Sharma P, Singh GK, Verma P. A case of well-differentiated squamous cell carcinoma in vulval lichen sclerosus. J Mar Med Soc 2022;24, Suppl S1:129-31

How to cite this URL:
Das P, Arora S, Sharma P, Singh GK, Verma P. A case of well-differentiated squamous cell carcinoma in vulval lichen sclerosus. J Mar Med Soc [serial online] 2022 [cited 2022 Aug 9];24, Suppl S1:129-31. Available from: https://www.marinemedicalsociety.in/text.asp?2022/24/3/129/342371




  Introduction Top


Lichen sclerosus (LS) is a chronic progressive inflammatory disease of genitalia seen in both males and females causing depigmentation, fibrosis, and scarring with potential for malignancy-most common of which is squamous cell carcinoma.[1] Women usually present with progressive vulvar pruritus and tightening of skin leading to dyspareunia while men complain of white fibrotic plaques or macules on glans and prepuce associated with difficulty retracting prepuce, pain, bleeding, and ulceration on coitus.[2] This case is presented for premalignant potential of LS et atrophicus to reinforcement and the need to follow-up such patients.


  Case Report Top


A 42-year-old married female presented with slowly progressive whitish discoloration and tightness of vulva of last 12-year duration and a painless growth on the clitoris for 6-week duration. She also complained of pain associated with coitus for last 3-year duration which was progressive and affecting quality of life. She noticed a small growth on the clitoris for last 6 weeks which was not associated with any pain, itching, discharge, or bleeding. The growth doubled in size by the time she sought medical consultation. Her general and systemic examination were within normal limits. The examination of vulva revealed a small 1 cm × 0.5 cm growth arising out of the clitoris with scaly and rough surface. It was firm and nontender. Vulva showed atrophy with whitish discoloration with difficulty in pinching the skin suggestive of underlying fibrosis [Figure 1]. These areas of atrophy and fibrosis extended from vulva into vagina as well as to mons pubis and perineum. However there was no regional lymphadenopathy. Rest of the dermatological examination was normal. Serological tests for human immunodeficiency virus 1 and 2 and Borrelia were negative. An excision biopsy was performed from the growth with suspicion of malignancy and condyloma acuminata in a case of chronic LS. The epidermis showed that full-thickness dysplasia in the epidermis was well as underlying dermis [Figure 2]a. Subepidermal tissue showed invasive malignant cells arranged in islands with surrounding desmoplastic reaction and squamous differentiation with abundant keratin pearls. Discrete malignant cells were round to polygonal in shape as well as irregularly sized with moderate to abundant amount of cytoplasm and pleomorphic nuclei with vesicular chromatin and increased mitoses [Figure 2]b. The patient was diagnosed as a case of well-differentiated squamous cell carcinoma in a known case of LS. She was referred to gynecologist for further evaluation and management.
Figure 1: A firm nontender nodule roughly measuring 1 cm × 0.5 cm on clitoris. There is atrophy of vulva with whitish discoloration suggestive of underlying lichen sclerosus.

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Figure 2: (a) Shows invasive malignant cells arranged in islands with squamous differentiation and surrounding desmoplastic reaction with abundant keratin pearls (H and E, ×10). (b) Discrete malignant cells were irregular in size and shape with pleomorphic nuclei and increased mitoses (H and E, ×40)

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  Discussion Top


LS (erstwhile LS et atrophicus) kraurosis vulvae or its analogous nosological entity balanitis xerotica obliterans in males is a chronic inflammatory dermatosis of uncertain etiology with premalignant potential.[3] Some LS lesions instead of atrophy may have hypertrophy and due to this reason, the suffix “et atrophicus” is not used anymore.[2] LS can affect all age groups, from infancy to elderly, but is most prevalent in uncircumcised middle-aged women and men. It is thought to be caused by built up of secretions and debris between prepuce and glans penis and around vulva leading to chronic inflammation resulting in fibrosis. Postmicturition dribbling and micro-incontinence has been hypothesized as one of the major etiologies leading to increased prevalence seen in uncircumcised men.[4] The involvement of peri-anal area in addition to peri-vulvar area in females in classic “figure of 8” or “hourglass” pattern is believed to be due to unhindered incontinence of urine from vulva to perineum and then to peri-anal area which is shielded in males by scrotum.[1] Reported associations of LS with ileostomy, urostomy, and hypospadias further indicate that irritation and moisture factor in the etiology of LS.[5],[6] Farrell et al. demonstrated increased levels of tumor necrosis factor, interferon gamma, and interleukin-1 in vulval LS lesions.[7] LS is also hypothesized to be associated with obesity, smoking, and cardiovascular disease.[8] Women present with areas of insidious onset of whitish discoloration in and around vulva associated with itching and later slowly progressive tightness.[9] The presence of areas of red as well as white areas together may give rise to mottled appearance. It also may be associated with significant dyspareunia in women and priapism in men affecting quality of life.[8] Urinary symptoms of poor stream, dysuria, and urinary obstruction are seen predominantly in males due to involvement of urethra.[9] Histopathologically, there is atrophy of surface epithelium with flattening of rete ridges. Hyperkeratosis is accompanied with keratin plugging. In early stages, there is subepidermal lymphedema which progresses to loss of elastic tissue and homogenization of collagen in upper third of the dermis.[7] The British Journal of Dermatology guidelines recommend daily use of potent topical corticosteroids for adults as first-line therapy for LS.[10] Once under remission, patients should be under six monthly follow-up and reintroduction of topical corticosteroids only on relapse or else maintenance therapy by once weekly application of topical steroids.


  Conclusion Top


It is important to educate the patients with LS about the disease course and its malignant potential and the vital role of regular self-examination and follow-up. Our case adds to the existing literature that LS is indeed a premalignant lesion.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal.

Acknowledgments

We sincerely thank Department of Pathology, Army Hospital Research and Referral, who helped us in the diagnosis and management of the case.

Financial support and sponsorship

Nil.

Conflict of interest

There are no conflicts of interest.



 
  References Top

1.
Nair PA. Vulvar lichen sclerosus et atrophicus. J Life Health 2017;8:55-62.  Back to cited text no. 1
    
2.
Marfatia Y, Surani A, Baxi R. Genital lichen sclerosus et atrophicus in females: An update. Indian J Sex Transm Dis AIDS 2019;40:6-12.  Back to cited text no. 2
    
3.
Nguyen AT, Holland AJ. Balanitis xerotica obliterans: An update for clinicians. Eur J Pediatr 2020;179:9-16.  Back to cited text no. 3
    
4.
Fergus KB, Lee AW, Baradaran N, Cohen AJ, Stohr BA, Erickson BA, et al. Pathophysiology, clinical manifestations, and treatment of lichen sclerosus: A systematic review. Urology 2020;135:11-9.  Back to cited text no. 4
    
5.
Figler BD, Gomella A, Hubbard L. Staged urethroplasty for penile urethral strictures from lichen sclerosus and failed hypospadias repair. Urology 2018;112:222-4.  Back to cited text no. 5
    
6.
Al-Niaimi F, Lyon C. Peristomal lichen sclerosus: The role of occlusion and urine exposure? Br J Dermatol 2013;168:643-6.  Back to cited text no. 6
    
7.
Farrell AM, Dean D, Millard PR, Charnock FM, Wojnarowska F. Cytokine alterations in lichen sclerosus: An immunohistochemical study. Br J Dermatol 2006;155:931-40.  Back to cited text no. 7
    
8.
Hofer MD, Meeks JJ, Mehdiratta N, Granieri MA, Cashy J, Gonzalez CM. Lichen sclerosus in men is associated with elevated body mass index, diabetes mellitus, coronary artery disease and smoking. World J Urol 2014;32:105-8.  Back to cited text no. 8
    
9.
Das S, Tunuguntla HS. Balanitis xerotica obliterans – A review. World J Urol 2000;18:382-7.  Back to cited text no. 9
    
10.
Lewis FM, Tatnall FM, Velangi SS, Bunker CB, Kumar A, Brackenbury F, et al. British Association of Dermatologists guidelines for the management of lichen sclerosus, 2018. Br J Dermatol 2018;178:839-53.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]



 

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