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Congenital self-healing reticulohistiocytosis: concern for a poor prognosis
Larissa Larsen1, Melissa Reyes Merin, Thomas Konia
1School of Medicine, University of California Davis, Sacramento, California, USA.
Insights
Congenital self-healing reticulohistiocytosis (CSHRH) can relapse and involve organs, despite its traditional view as benign. Early diagnosis and monitoring are crucial, especially in infants, to prevent severe outcomes.
Area of Science:
- Dermatology
- Pediatric Pathology
- Histiocytosis
Background:
- Congenital self-healing reticulohistiocytosis (CSHRH) is a rare Langerhans cell histiocytosis variant.
- Traditionally viewed as benign, CSHRH carries risks of mortality (3%) and relapse (10%).
Purpose of the Study:
- To review CSHRH cases since 1973.
- To highlight CSHRH presentations, relapse rates, and systemic involvement, particularly in infants.
- To emphasize the importance of considering CSHRH in neonates with congenital skin lesions.
Main Methods:
- Extensive literature review of CSHRH cases.
- Analysis of case presentations, focusing on infants within the first year of life.
- Evaluation of relapse and systemic involvement patterns.
Main Results:
- CSHRH exhibits diverse presentations.
- High rates of relapse and systemic involvement are observed, especially in infants.
- Cases in the first year of life show increased risk.
Conclusions:
- CSHRH requires consideration in neonatal differential diagnoses for congenital skin eruptions.
- Prompt diagnosis and management of systemic CSHRH are vital for reducing adverse outcomes.
- Increased patient monitoring is recommended, particularly during the first year of life.
Abstract:
Congenital self-healing reticulohistiocytosis (CSHRH) is a rare type of Langerhans cell histiocytosis with potential for relapse and systemic involvement. Whereas CSHRH was traditionally considered a benign disease, there is an approximately 3 percent risk of mortality and a 10 percent chance of relapse. This article, using an extensive review of cases since Hashimoto and Pritzker first described the condition in 1973, highlights the various presentations of CSHRH and reveals high rates of relapse and systemic involvement in cases that specifically address features of CSHRH occurring within the first year of life. The findings from this review will highlight the importance of considering LCH in the differential diagnosis when evaluating a neonate with congenital skin eruptions. Timely diagnosis of CSHRH and treatment of systemic involvement may decrease the likelihood of adverse outcomes. These patients may require closer follow-up and monitoring than previously recommended, especially in the first year of life when relapses and systemic involvement occur most frequently.
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