Childhood Lichen Sclerosus—A Challenge for Clinicians

Abstract

Childhood lichen sclerosus (LS) is a rare and often misdiagnosed inflammatory dermatitis with an unpredictable course. The complications of LS are architectural changes of the vulva; malignant transformation is possible. The objective of our study was to define the background and the long-term course of childhood LS. A registery study identified 44 children with LS treated at Tampere University Hospital, Tampere, Finland, from 1982 to 2010. A questionnaire was sent to the identified patients and 15 responded. The clinical depiction of LS varied significantly. LS was diagnosed in only 16% of the patients at the referring unit. Autoimmune disorders were observed in 6 of the 44 patients. High prevalences of Turner's syndrome (2/44) and kidney disease (2/44) were noted. The majority of the patients were treated with topical corticosteroids. Eight developed architectural changes of the vulva. The questionnaire revealed that three of six patients who were asymptomatic at the end of the registery study follow-up experienced a recurrence of symptoms. None of them were undergoing follow-up. Nine of the 15 patients reported reduced quality of life. Childhood LS is a heterogeneous disease with a remarkable effect on quality of life. The misdiagnosis of childhood LS is common. The association between LS and autoimmune diseases should be noted. The high prevalence of Turner's syndrome raises questions regarding the influence of low estrogen levels on the development of LS. The prognosis cannot be predicted, so long-term follow-up is recommended. New tools for diagnosis and surveillance are needed.

Cutaneous Findings Mistaken for Physical Abuse: Present but Not Pervasive

“…This contrasts with studies of children with congenital factor deficiencies who are frequently initially evaluated for abuse and sometimes initially diagnosed as abused . Lichen sclerosis, which is commonly described as a cutaneous mimic of sexual abuse, was not identified in this cohort of children, which is probably because the children were referred for physical abuse evaluations .…”

Contact Allergy and Vulvar Lichen Sclerosus et Atrophicus

“…These women are severely affected; studies indicates that the disease has a large effect on quality of life and many suffer from sexual dysfunction as well [5,[10][11][12]. Even a small improvement will make a difference for the women and therefore we do think that patch testing these patients is important.…”

“…Lichen sclerosus et atrophicus is a chronic inflammatory disorder that can involve the skin, the mucosa and nails with a predilection for the anogenital area [1,2]. It occurs in both sexes and can begin at any age; actually it has been described as an increasingly common problem in children [3][4][5]. There is a first peak in prepubertal girls and a second peak in postmenopausal women [1,2,6].…”

“…Pruritus, pain and dyspareunia are common symptoms and as a result there may be a complete preclusion of sexual intercourse. Women and young girls with the disease describe restrictions in their lifestyle and a reduced quality of life [5,[10][11][12].…”

“…In women the scarring atrophy causes gradual destruction of normal vulvar architecture with resorption of the labia minora, narrowing of the introitus and burying of the clitoris [1,2]. Constrictions of the urethra and anus may lead to retention of urine, painful defaecation and constipation [1,2,5]. Pruritus, pain and dyspareunia are common symptoms and as a result there may be a complete preclusion of sexual intercourse.…”