Abstract
Background:Cutaneous langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation of cells with phenotypical characteristics of Langerhans cells. Although some cases spontaneously resolve, no consistent variables have been identified that predict which cases will manifest with systemic disease later in childhood.Methods:A systematic review (Pubmed, Embase, Cochrane database and all published abstracts from 1946-2018) was undertaken to collate all reported cases of cutaneous LCH in the international literature. This study was registered with PROSPERO (CRD42016051952). Descriptive statistics and correlation analyses were undertaken. Bias was analyzed according to GRADE criteria.Results:A total of 83 articles encompassing 128 cases of cutaneous LCH were identified. Multiple lesions were weakly associated with an increased length of survival (R=0.304 (p<0.05)), Worse prognosis was associated with internal organ involvement with a statistically significant chi squared statistic (χ2=14.96, 2DF p<0.001) and an elevated odds ratio ((OR)= 12.30 95% CI=2.67-56.74). Vesicular lesions (OR=10.8 95% CI=2.83-41.26), but not ulceration (OR=0.53 95% CI 0.12-2.05) were associated with greater risk of mortality.Conclusions:Congenital and neonatal LCH most commonly presents as multiple lesions in multiple anatomical sites at birth. Significant differences, including the associations of mortality with lesion morphology and number were seen in this neonatal cohort compared to overall pediatric LCH. These findings require validation in a large prospective cohort.
Highlights
Cutaneous langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation of cells with phenotypical characteristics of Langerhans cells
We present a systematic review of case reports of cutaneous congenital and neonatal Langerhans cell histiocytosis (LCH)
The descriptive characteristics in this review significantly differ from descriptions of overall pediatric LCH, highlighting the clinical differences between these entities
Summary
Cutaneous langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation of cells with phenotypical characteristics of Langerhans cells. Consensus guidelines[1] state that most cases of cutaneous LCH spontaneously regress but some cases do progress to multisystem disease[1]. It is unclear whether cutaneous LCH is merely clinically more identified and often precedes diagnosis of internal disease. This would suggest that widespread screening of cases of cutaneous LCH may produce leadtime bias in the survival rates of individuals with multisystem LCH with cutaneous involvement, an issue which to date has not been explored. It would have been helpful to have more background information as to the previously documented frequency of skin-limited LCH, as the paper doesn't clearly provide this (i.e. How common is skin-limited LCH that doesn't progress (or regresses))
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