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- W2100037794 abstract "<h3>Objectives</h3> To describe clinical and immunohistochemical findings in patients with cutaneous Langerhans cell histiocytosis (LCH) beginning in the first 3 months of life and to define predictors of disease evolution. <h3>Design</h3> Observational retrospective survey from July 15, 1989, to April 30, 2007. <h3>Setting</h3> Referral center in pediatric dermatology. <h3>Patients</h3> Thirty-one patients with a diagnosis of cutaneous LCH in the first 3 months of life and no previous visceral LCH. <h3>Main Outcome Measures</h3> Cutaneous lesion characteristics, regulatory T-lymphocyte density, and E-cadherin expression were assessed. Data were compared between the patient groups with self-regressive vs non–self-regressive forms of cutaneous LCH. Pathologic analysis was performed blinded to patient group. <h3>Results</h3> Self-regressive cutaneous LCH was found in 21 patients and non–self-regressive cutaneous LCH in 10 patients. Monolesional forms, necrotic lesions, hypopigmented macules at presentation, and distal topography of limb lesions were seen only in patients with self-regressive cutaneous LCH. Regulatory T-lymphocyte density correlated with interleukin 10 expression in lesions (<i>r</i> = 0.77,<i>P</i> = .003) but was not predictive of disease evolution. E-cadherin expression by Langerhans cells was found in 7 patients with disease limited to the skin whether self-regressive or not. One patient with secondary disseminated disease showed loss of E-cadherin expression in Langerhans cells. <h3>Conclusions</h3> Some morphologic traits of skin lesions can orient the diagnosis to a self-regressive form of cutaneous LCH. Regulatory T-lymphocyte density does not seem to be predictive of disease evolution. E-cadherin expression seems to be an indicator of limited skin disease but not of disease regression. Additional immunohistochemical study is required to confirm these data." @default.
- W2100037794 created "2016-06-24" @default.
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- W2100037794 date "2010-02-01" @default.
- W2100037794 modified "2023-09-26" @default.
- W2100037794 title "Neonatal and Early Infantile Cutaneous Langerhans Cell Histiocytosis" @default.
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- W2100037794 doi "https://doi.org/10.1001/archdermatol.2009.360" @default.
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