|Year : 2022 | Volume
| Issue : 3 | Page : 129-131
A case of well-differentiated squamous cell carcinoma in vulval lichen sclerosus
Pankaj Das1, Sandeep Arora1, Pragya Sharma2, Gautam Kumar Singh1, Prachi Verma1
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 Submission||28-Aug-2021|
|Date of Decision||18-Nov-2021|
|Date of Acceptance||29-Nov-2021|
|Date of Web Publication||01-Apr-2022|
(Dr) Pankaj Das
Department of Dermatology, Base Hospital Delhi Cantt and Army College of Medical Sciences, New Delhi - 110 010
Source of Support: None, Conflict of Interest: None
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
| Introduction|| |
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. 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. This case is presented for premalignant potential of LS et atrophicus to reinforcement and the need to follow-up such patients.
| Case Report|| |
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|| |
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. Some LS lesions instead of atrophy may have hypertrophy and due to this reason, the suffix “et atrophicus” is not used anymore. 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. 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. Reported associations of LS with ileostomy, urostomy, and hypospadias further indicate that irritation and moisture factor in the etiology of LS., Farrell et al. demonstrated increased levels of tumor necrosis factor, interferon gamma, and interleukin-1 in vulval LS lesions. LS is also hypothesized to be associated with obesity, smoking, and cardiovascular disease. Women present with areas of insidious onset of whitish discoloration in and around vulva associated with itching and later slowly progressive tightness. 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. Urinary symptoms of poor stream, dysuria, and urinary obstruction are seen predominantly in males due to involvement of urethra. 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. The British Journal of Dermatology guidelines recommend daily use of potent topical corticosteroids for adults as first-line therapy for LS. 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|| |
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.
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
Conflict of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]