Diagnosis and Management of Lichen Sclerosus in a Young Female
Published Date: August 20, 2017
Diagnosis and Management of Lichen Sclerosus in a Young Female
Joyce Cheng1*, Erica Jarcaig2, Gareth Edwards3, and Pranav Patel4
1MHA, Saint James, School of Medicine, Albert Lake Dr, The Quarter, A-1 2640, Anguilla, USA
2Saint James, School of Medicine, Albert Lake Dr, The Quarter, A-1 2640, Anguilla, USA
3Atlantic University, School of Medicine, Gros Islet, St Lucia, USA
4MD, Internal Medicine, 12800 S. Ridgeland Ave, Palos Heights, IL 60463, USA
*Corresponding author: Joyce Cheng, MHA, Saint James, School Of Medicine, 15723 Chesterfield lane, Orland Park, IL 60462, USA, Tel: 951-902-7628; E-mail: firstname.lastname@example.org
Citation: Cheng J, Jarcaiga E, Edwards G, Patel P(2018) Diagnosis and Management of Lichen Sclerosus in a Young Female. J Derma Pigm Res 2(1):101.
This case report involves a patient who has been diagnosed with lichen sclerosus. Lichen sclerosus, also known as white spot disease, is a chronic inflammatory skin disease that mainly affects adult women, occurring less commonly in men (referred to as lichen planus) and rare in children. Lichen sclerosus is an incurable disease and mostly require managements of the symptoms only. Other manifestations may include hypothyroidism, diabetes, and vitiligo. The purpose of this case study is to discuss the clinical manifestations of Lichen sclerosis, diagnosis, treatment and management, complications, and prognosis of patients suffering from the disease.
Keywords: Lichen Sclerosus; Symptoms; White spot disease; Skin disease
Lichen sclerosus, also known as white spot disease, is a chronic inflammatory skin disease that mainly affects adult women, less commonly in men and rare in children. Any skin area may be affected however; lichen sclerosus is most common in the anogenital areas. Extragenital lesions may also occur including upper arms, chest areas, and rarely, the oral mucosa causing itching, discomfort, pain and tearing of the skin [1,6,7].
Initial symptoms include small white spots on the affected area (Figure 1). The white spots are often smooth and shiny which progresses into larger, blotchy and wrinkled patches with purple discoloration (Figure 2). Painful fissures and tears can occur with sexual intercourse and with defecation. Histological features include marked thinning of the epidermis, degeneration of basal cells, excessive keratinization, sclerotic changes of the superficial dermis and band-like lymphocytic infiltrate of the underlying dermis (Figure 3) [6,7,9].
A 33 year old gravida 2, para 2 Indian female presented at the clinic with concerns about long-term use of topical steroid cream for her diagnosis of Lichen sclerosus. Patient immigrated from India in 1989 at the age of 7. At the age of 10, the patient noticed areas of hypopigmentation of her skin on her knees and ankles bilaterally and was diagnosed with vitiligo. Patient noticed the white spots were spreading more after her second pregnancy in 2014. At this time, patient noticed white spots on her vulva that were pruritic. Patient visited gynecologist and was diagnosed with lichen sclerosus by clinical observation. Biopsy was performed in April 2016 to confirm the diagnosis. During physical examination on the 1st of February 2017, hypopigmentation was noted on several areas of her body including: the lips, the distal aspect of her fingers, the dorsum of her hands, palmar aspect of her wrists, elbows, and her neck. Large patch of hypopigmentation also present on bilateral lower extremities.
A biopsy of two specimens was taken in 04/20/2016:
Specimen A) skin right vulva; specimen B) skin left lower vulva.
After evaluation diagnosis:
Specimen A: Squamous hyperplasia and hyperkeratosis. No dysplasia identified
Specimen B: Lichen Sclerosus Et Atrophicus. No dysplasia identified.
No further excision of biopsy site was needed due to lack of metaplasia. Patient could then continue the application of topical corticosteroid Clobetasol propionate as advised. Other underlying autoimmune disorders were excluded based on negative antinuclear antibody (ANA) in lab findings.
Lichen Sclerosus (LS) is an inflammatory skin condition characterized by the presence of smooth, white plaques or macules. Over time, these lesions may coalesce to produce areas of skin that resemble parchment. Lichen Sclerosus may affect any area of skin however, it most commonly involves skin of the vulva, foreskin of the penis or skin around the anus. We are presenting a case of LS affecting the vulva of a 33-year-old woman, which is rare for this condition.
The clinical manifestations of LS may vary depending on location and severity of disease. The case presented manifested with pruritic white lesions of the vulva and dyspareunia. However, more severe cases may also present with bleeding, blistering or ulcerated lesions, blotchy and wrinkled patches, and easy bruising or tearing of the skin involved. Gold standard for diagnosis is excisional biopsy and histopathological examination. Lichen sclerosus is characterized by marked thinning of the epidermis, degeneration of basal cells, excessive keratinization, sclerotic changes of the superficial dermis and band-like lymphocytic infiltrate in the underlying dermis.
Etiology of LS is not exactly known however, some studies have found some an association with hormone imbalance such as in postmenopausal women. Presence of activated T cells in the subepithelial, inflammatory infiltrate and increased frequency of autoimmune disorders in affected women suggest an autoimmune reaction is involved. Other etiologies of the development of Lichen Sclerosus and Vitiligo can be explored in relation to previous bacterial infection such as Borrelia burgdorferi. However, additional studies are necessary to conclude a causal association between bacterial infection and the prevalence of Lichen Sclerosus . Differential diagnosis includes lichen planus, low estrogen level, vitiligo, pemphigoid, and hemorrhoids [1,7,9].
Vitiligo is a long-term skin problem that results in patches of white skin depigmentation in a symmetrical pattern. Depigmentation occurs when the melanocytes of the area die off and it can affect any race, age and gender .
Vitiligo can affect any parts of the body, including the eyes, inside of the mouth, hair, and any parts of the skin. The exact causes of vitiligo are unknown, however, previous studies have found close associations of Vitiligo with autoimmune disorder, where the patient’s immune system become overactive and destroys the melanocytes. Other causes of Vitiligo include family inheritance, chemical exposure, stressful events, as well as viral infections .
Vitiligo is not transmittable and is not life threatening. Hence, the management and treatment for Vitiligo is mostly to restore skin color, thickness and strength. Most common option include cosmetic to cover up white patches, corticosteroids are also used help suppress immune system to help improve skin repigmentation. Unconventional treatments include vitamins and minerals. Previous studies have shown improvements after 3-6 months treatment with folic acid and vitamin B12, including sun exposure . Over 50% of the patients showed repigmentation and 6 out of the 100 participants showed total repigmentation after the treatment .
Diagnosis: Diagnosis may be made clinically but is confirmed with a biopsy. Tests such as estradiol/estrogen levels, antinuclear antibody, as well as other high sensitivity autoimmune antibody tests can also be done to exclude other differential diagnosis.
Complications: Women that have Lichen sclerosus on the vulva have a slightly increased risk of developing squamous cell carcinoma (<5%) and constant use of topical corticosteroids may help reduce the risk . However, lichen sclerosus may cause thinning of the skin and result in tears and abrasions, which can lead to excess fibrosis resulting in a shrinkage of the vaginal opening (Figure 4). Squamous cell carcinoma is predominantly associated with female genital lichen sclerosus but is not associated with extragenital lichen sclerosus. Other malignancy that is involved with lichen sclerosus includes verrucous carcinoma, melanoma, basal cell carcinoma, and merkel cell carcinoma . Associations between lichen sclerosus and penile squamous cell carcinoma has also been reported, especially in uncircumcised men. Lichen sclerosus can also cause scarring and introital narrowing in affected men, resulting in dyspareunia and difficulty with micturition often requiring surgery .
Treatment and Management: Lichen sclerosus on the upper body and extremities does not require treatment as the lesion resolves in time. However, lichen sclerosus of the genitalia often causes discomfort and itchiness and is treated with topical steroids (clobetasol and mometasone ointment). Men with lichen sclerosus are treated surgically (circumcision or foreskin preputioplasty) and are less likely to experience recurrences [3,4].
Regular follow up is important since long-term uses of steroids because thinning and atrophy of the skin and increase risk of yeast infections. Other treatment options include retinoids and immune modulators. Long term follow up for the patient included a regiment of Hydroxocobalamin intramuscular injections on a time frame of one per week for a month, then one per month for three months followed by one per trimester. Patient was also advised of 5,000 units Vitamin D3 and follow-up with dermatologist. Patient was advised to see rheumatologist if any bone/joint issues arise. Patient was told Tacrolimus should be considered as treatment refractory to current methods.
Outcome of the Case: After a month of treatment with Clobetasol, daily vitamin D supplements, and Vitamin B12 injection, patient experienced decreased pruritus on her hypopigmented areas. Hypopigmented patches also showed minor repigmentation of areas around her chest and neck area. Patient is suggested to continue with current regimen.
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Copyright: © 2018 Cheng J This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.