PDF(Sci-hub)
Abstract：
Lichen sclerosus is a chronic skin disease, mainly localised at the introitus and perineum. When the condition remains untreated, gradual atrophy of skin structures leads to permanent scarring, making early diagnosis and treatment crucial. We reviewed all patients diagnosed with lichen sclerosus presenting to a tertiary referral centre for paediatric and adolescent gynaecology between January 2011 and December 2015 to assess disease presentation and response to treatment. We identified 15 cases, with a mean age at diagnosis of 8.8 years. Their main presenting symptoms were vulvar pruritus and vulvar soreness. Seven girls had already atrophic changes, and in four girls, this amounted to clitoral phimosis, labial resorption or labial adhesion formation. The median delay in diagnosis was 7 months. Thirteen patients received local treatment with potent corticosteroids, responding well to treatment. However, 4 girls relapsed within 2 to 36 months. Two adolescents required surgical treatment, one because of urinary retention and the second because of dyspareunia caused by clitoral entrapment.
CONCLUSIONS: There was a delay in diagnosis in most patients and this resulted in irreversible genital skin changes, which would have been preventable, had treatment been instituted promptly. The response to treatment with local corticosteroids was usually effective, leading to both symptom alleviation and prevention of disease progression. Atrophic changes and skin complications however were not reversed. What is Known: • Lichen sclerosus affects women of all ages, including girls, particularly prior to adolescence. • Lichen sclerosus responds well to local corticosteroid treatment. What is New: • In the majority of patients with lichen sclerosus there was a long delay between onset of symptoms and diagnosis. • Nearly half of the children diagnosed with lichen sclerosus had irreversible atrophic genital skin changes at the time of first presentation. These changes may have been prevented by a timely diagnosis and intervention.
CONCLUSIONS: There was a delay in diagnosis in most patients and this resulted in irreversible genital skin changes, which would have been preventable, had treatment been instituted promptly. The response to treatment with local corticosteroids was usually effective, leading to both symptom alleviation and prevention of disease progression. Atrophic changes and skin complications however were not reversed. What is Known: • Lichen sclerosus affects women of all ages, including girls, particularly prior to adolescence. • Lichen sclerosus responds well to local corticosteroid treatment. What is New: • In the majority of patients with lichen sclerosus there was a long delay between onset of symptoms and diagnosis. • Nearly half of the children diagnosed with lichen sclerosus had irreversible atrophic genital skin changes at the time of first presentation. These changes may have been prevented by a timely diagnosis and intervention.
摘要：
硬化性苔藓是一种慢性皮肤病,主要位于鼻部和会阴部。当病情未得到治疗时，皮肤结构的逐渐萎缩导致永久性疤痕，早期诊断和治疗至关重要。我们回顾了2011年1月至2015年12月期间到三级儿科和青少年妇科转诊中心诊断为硬化性苔藓的所有患者，以评估疾病的表现和对治疗的反应。我们确定了15例,诊断时的平均年龄为8.8岁。他们的主要症状是外阴瘙痒和外阴酸痛。七个女孩已经出现了萎缩性变化，在四个女孩中，这相当于阴蒂包茎，阴唇吸收或阴唇粘连形成。诊断的中位延迟为7个月。13例患者接受强效皮质类固醇的局部治疗，对治疗反应良好。然而,4名女孩在2至36个月内复发。两名青少年需要手术治疗，一个原因是尿潴留，第二个原因是阴蒂压迫引起的性交困难。
结论：大多数患者的诊断延迟，这导致了不可逆的生殖器皮肤变化，本来是可以预防的，治疗迅速开始。对局部皮质类固醇治疗的反应通常是有效的，导致症状缓解和预防疾病进展。然而，萎缩性变化和皮肤并发症并未逆转。什么是已知的:•硬化苔藓影响所有年龄段的妇女,包括女孩,特别是在青春期之前。•硬化性苔藓对局部皮质类固醇治疗反应良好。新增内容:•在大多数患有硬化性苔藓的患者中,在症状发作和诊断之间存在长的延迟。•将近一半被诊断为硬化性苔藓的儿童在首次就诊时出现了不可逆的萎缩性生殖器皮肤变化。这些变化可以通过及时的诊断和干预来预防。
结论：大多数患者的诊断延迟，这导致了不可逆的生殖器皮肤变化，本来是可以预防的，治疗迅速开始。对局部皮质类固醇治疗的反应通常是有效的，导致症状缓解和预防疾病进展。然而，萎缩性变化和皮肤并发症并未逆转。什么是已知的:•硬化苔藓影响所有年龄段的妇女,包括女孩,特别是在青春期之前。•硬化性苔藓对局部皮质类固醇治疗反应良好。新增内容:•在大多数患有硬化性苔藓的患者中,在症状发作和诊断之间存在长的延迟。•将近一半被诊断为硬化性苔藓的儿童在首次就诊时出现了不可逆的萎缩性生殖器皮肤变化。这些变化可以通过及时的诊断和干预来预防。
