Vulvar Lichen Sclerosus Is Very Frequent in Women With Turner Syndrome

Chakhtoura, Zeina; Vigoureux, Solène; Courtillot, Carine; Tejedor, Isabelle; Touraine, Philippe

doi:10.1210/jc.2013-3480

Cited by 18 publications

(14 citation statements)

References 4 publications

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“…Dermatological assessment revealed V and P in 2.2 and 1.5% of the studied TS girls. Unlike in the adult TS population (14), AA and LS were diagnosed in only one patient (0.7%) each.…”

Section: Discussionmentioning

confidence: 68%

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Autoimmunity Predisposition in Girls With Turner Syndrome

et al. 2019

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Background: Turner Syndrome is associated with an increased risk of autoimmune diseases, such as autoimmune thyroiditis, coeliac disease, type 1 diabetes mellitus, inflammatory bowel disease, alopecia areata, or vitiligo. The presence of isochromosome iXq and exposure to estradiol may contribute to the development of the autoimmune process. The aim of this study was to determine the prevalence of autoimmune diseases in a group of TS patients and to assess the impact of karyotype and puberty on the development of autoimmune diseases. Patients and Methods: The analysis encompassed clinical and biochemical data of 134 patients treated between 2001 and 2018. All the patients were examined for autoimmune disease symptoms and tested for the presence of antithyroperoxidase (anti-TPO) and antithyreoglobulin (anti-TG) antibodies. In 73 of the patients, anti-transglutaminase (anti-tTG) antibodies were measured. Thyroid function was assessed by measuring TSH and fT4 levels. Results: The mean follow-up was 5.7 ± 3 years. An autoimmune disease was diagnosed in 46 (34.3%) patients: 39 (29.1%) had only one disorder, whilst 7 (5.2%) presented two disorders. The most common disorder, observed in 40 (29.9%) patients, was thyroid autoimmunity. Hashimoto disease was diagnosed in 20 (14.9%) patients. Of the 73 patients tested for coeliac disease, 4 (5.5%) had anti-tTG and 2 (2.7%) presented overt coeliac disease. Vitiligo was diagnosed in 3 (2.2%) patients, type 1 diabetes mellitus or psoriasis were diagnosed in 2 (1.5%) patients, whilst alopecia areata or lichen sclerosus were diagnosed in 1 (0.7%) patient. The impact of karyotype or estradiol exposure on developing autoimmune diseases were not statistically significant. Conclusions: Our study showed a higher incidence of autoimmune diseases in TS, which is in line with the literature; however, the impact of iXq, or spontaneous/inducted puberty was not confirmed.

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“…Dermatological assessment revealed V and P in 2.2 and 1.5% of the studied TS girls. Unlike in the adult TS population (14), AA and LS were diagnosed in only one patient (0.7%) each.…”

Section: Discussionmentioning

confidence: 68%

“…Alopecia areata is reported to affect TS patients 3 times more frequently than the general population (13). Chakhtoura et al reported lichen sclerosus in 17.3% of their TS patients (14).…”

Section: Introductionmentioning

confidence: 98%

Autoimmunity Predisposition in Girls With Turner Syndrome

et al. 2019

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“…There were two patients (8%) in our cohort with a diagnosis of Turner syndrome (TS). LS has a reported prevalence of 17% in the TS population . Like LS, TS has a strong association with autoimmune disorders .…”

Section: Discussionmentioning

confidence: 99%

Paediatric vulval lichen sclerosus: a retrospective study

Ismail

Owen

2019

Clin Exp Dermatol

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Summary Background Lichen sclerosus (LS) is a chronic inflammatory dermatosis with a predilection for the anogenital region, which mainly affects prepubertal girls and postmenopausal women. The cause is unknown, but a number of potential aetiological factors have been identified. Aim To examine a cohort of patients with prepubertal‐onset vulval LS (VLS) and assess baseline characteristics, clinical presentation, potential precipitating and predisposing factors, and response to treatment. Methods Data were collected from case notes on patients aged < 18 years diagnosed with prepubertal‐onset VLS attending a specialist vulval dermatology service. Data included clinical presentation, comorbidities, family history, therapy and response to treatment. Results In total, 26 paediatric patients were identified. The median age at onset of symptoms was 5 years (range 2–8.5 years). Many previously identified potential aetiological factors for the development of VLS were identified, including family history, trauma, autoimmune disease and hormonal factors. A significant proportion of patients had a history of urinary tract symptoms, including incontinence and urinary tract infection. Most patients responded well to a standard course of induction topical therapy followed by maintenance therapy, but some, including three patients with ongoing urinary incontinence and three postpubertal patients, continued to have active disease. Conclusion A detailed assessment is essential in patients with VLS so that potential predisposing factors and comorbidities can be identified and managed. Urinary incontinence may be implicated in the development of paediatric VLS and may prevent adequate disease control. Paediatric VLS can persist through puberty, thus long‐term follow‐up is advised.

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“…This may have implications for future treatment, and for the prediction of the disease [ 14 ]. The literature also includes other genetic factors, i.e., Turner or Down syndrome [ 15 , 16 ].…”

Section: Etiopathogenesismentioning

confidence: 99%

Pediatric Vulvar Lichen Sclerosus—A Review of the Literature

Orszulak

Dulska

Niziński

et al. 2021

IJERPH

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Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition affecting the anogenital region, which may present in a prepubertal or adolescent patient. The most popular theories are its autoimmune and genetic conditioning, although theories concerning hormonal and infectious etiology have also been raised. The most common presenting symptoms of VLS is vulva pruritus, discomfort, dysuria and constipation. In physical examination, a classic “Figure 8” pattern is described, involving the labia minora, clitoral hood, and perianal region. The lesions initially are white, flat-topped papules, thin plaques, or commonly atrophic patches. Purpura is a hallmark feature of VLS. The treatment includes topical anti-inflammatory agents and long-term follow-up, as there is a high risk of recurrence and an increased risk of vulvar cancer in adult women with a history of lichen sclerosus. This article reviews vulvar lichen sclerosus in children and provides evidence-based medicine principles for treatment in the pediatric population. A systematic search of the literature shows recurrence of VLS in children. Maintenance regimens deserve further consideration.

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Vulvar Lichen Sclerosus Is Very Frequent in Women With Turner Syndrome

Cited by 18 publications

References 4 publications

Autoimmunity Predisposition in Girls With Turner Syndrome

Autoimmunity Predisposition in Girls With Turner Syndrome

Paediatric vulval lichen sclerosus: a retrospective study

Pediatric Vulvar Lichen Sclerosus—A Review of the Literature

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