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.

No abstract

SynopsisRituximab is an effective treatment for erosive vulvo–vaginal and oral lichen planus.

No abstract

The AMPK-related kinase NUAK2 is considered to play some role in melanoma development and progression. In our past studies, we showed that NUAK2 is a gene that has a prognostic significance in acral melanoma patients by verifying a public array comparative genomic hybridization (CGH) database and immunohistochemistry of 56 primary acral melanomas (PNAS, 2011) and that pharmacologic inhibition of CDK2 is sufficient to suppress the growth of NUAK2 amplified and PTEN-deficient melanoma cells (Cancer Res, 2015). This time, in order to validate the previous survival data with an increased number of cases, we performed immunohistochemical analyses using 87 specimens of acral melanomas. We also examined its relevance with DNA copy number gains revealed by metaphase CGH. We performed immunohistochemical analyses by using formalin-fixed, paraffin-embedded tissues from 87 acral melanomas and 40 Non-CSD melanomas, and performed metaphase CGH analyses from 12 acral melanomas and 6 Non-CSD melanomas. The Kaplan-Meier curves showed that both the relapse-free survival and the overall survival time of patients with acral melanomas expressing NUAK2 were significantly shorter than patients without the NUAK2 expression. The Cox regression model showed a significant difference in relapse-free survival in acral melanomas. All 6 cases with 1q32 gain, where NUAK2 resides, over-express NUAK2. These data demonstrate that over-expression of NUAK2 has a significant impact on the survival of acral melanoma patients.