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Cicatricial alopecias: Pathogenesis, classification, clinical features, diagnosis, and management
Published in Jerry Shapiro, Nina Otberg, Hair Loss and Restoration, 2015
Lichen sclerosus et atrophicus is a chronic inflammatory dermatosis that results in white plaques with epidermal atrophy. In rare cases, it presents with bullae, erosions, and scarring, including scarring alopecia [137,138].
Case 61
Published in Vincent J Palusci, Dena Nazer, Patricia O Brennan, Diagnosis of Non-accidental Injury, 2015
A 4-year-old girl presented with a 1-year history of genital and perianal pruritus. There was hypopigmentation of the labia majora, thickening to the clitoral hood, bruising to the labium minus on the right, and extension of the lesion to the perianal area (Image 61). Topical antifungal treatments had been tried without help. Sexual abuse was suspected based on the clinical findings. The child did not make any disclosure of abuse. What is the diagnosis?What are the treatment options?Are there any cancer risks?The diagnosis of this child’s condition is lichen sclerosus (LS; previously known as lichen sclerosus et atrophicus). LS is an inflammatory affliction of the genitals. It occurs mainly in females, and 10%–15% of cases occur before puberty. The average age of presentation is 5 years but children may present as early as 6 months.1 The main symptoms are irritation, itching, discharge, pain on urination, burning and bleeding (from the scratching). In boys, it can cause phimosis and difficulty voiding. The pruritus and itching cause most of the symptoms and signs such as lichenification, bruising and dyschromia. The bruising is often mistaken for child abuse. The involved skin is friable and may bleed which raises the concern of an acute trauma.2 The classic findings are the whitened interior of the labia, perineum and perianal skin making a ‘figure eight’. The classic distribution, symptoms and colour make the diagnosis. Biopsy is helpful in complex cases.Therapy for girls is high-potency topical steroid ointments or topical immune modulator ointments. The therapy of choice for phimosis in boys is circumcision.3Adult females with prolonged LS are at a low-level risk for squamous cell carcinoma of the perineal skin. This has not been seen in children.
Lichen sclerosus of the vulva
Published in Climacteric, 2021
Lichen sclerosus of the vulva (LSV) is seen frequently enough in general gynecological practice to warrant knowledge about diagnosis and treatment. It is a chronic, progressive, inflammatory epithelial disease that affects the inner aspects of the labia majora, labia minora, perineum, perianal area and clitoris, manifesting as glistening white or hypopigmented thin areas of the skin of the sites, which can be isolated or confluent, leading to skin atrophy. Alternatively, the skin may appear thickened and parch-like or leathery in appearance. Invariably, there are associated multiple ecchymoses, fissuring of posterior commissure and adhesions of the vaginal labia or clitoral hood. LSV rarely affects the vagina, but it can affect the urethral orifice. In about 15% of the women, extragenital sites may be affected, including the thighs, buttocks, anal cleft, upper trunk or axilla. Previously known as lichen sclerosus et atrophicus, vulval dystrophy, kraurosis or leukoplakia, in 1976 the International Society for the Study of Vulvovaginal Diseases decided to call the disease lichen sclerosus and it has remained as such ever since [1–3].
Lichen sclerosus of the oral mucosa: clinical and histopathological findings. Review of the literature and a case report
Published in Acta Odontologica Scandinavica, 2018
Anna-Maija Matela, Jaana Hagström, Hellevi Ruokonen
Hallopeau first described LS in the vulva in 1887 [3]. LS was earlier called lichen sclerosus et atrophicus, lichen albus and white spot’s disease. As atrophy is not always present in the lesions, the name was changed to LS in 1976 [4]. The prevalence of LS is 0.1–0.3% [5] and is more frequent among women. Women are 6–10 times more often affected by genital LS compared to men. 15–20% of patients with genital LS also have extragenital manifestations, while only 6% of LS patients have extragenital lesions exclusively [1,2]. While LS can appear at any age, LS is most commonly diagnosed among pre-pubertal or post-menopausal women [1]. The mean age of girls at the time of diagnosis is 6.7 years and of women 60 years [6,7]. Men are usually diagnosed between the ages of 30–40 years [1]. Approximately 10–15% of LS patients are children, and most are girls with genital-area lesions [8]. However, the typical LS patient is a postmenopausal Caucasian woman between the ages of 50–60 years.
Generalized lichen sclerosus et atrophicus combined with ankylosing spondylitis responding to secukinumab
Published in Scandinavian Journal of Rheumatology, 2023
Q Ye, K-J Chen, M Jia, L-J Deng, S Fang
Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory skin disease of unknown aetiology. It mainly affects the region of the anus and genitalia, but rarely occurs outside the genitals. The disease may be related to hormones, heredity, infection, trauma, and autoimmune diseases (1). Treatment includes topical corticosteroids, tacrolimus, and ultraviolet-A1 phototherapy. Secukinumab is an interleukin-17 (IL-17) inhibitor that has been shown to play a role in inhibiting fibrosis and has been approved for the treatment of ankylosing spondylitis (AS) (2). In this article, we report a case of generalized LSA complicated with AS responding to the IL-17 inhibitor secukinumab.