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Cutaneous Langerhans cell histiocytosis in children under one year
Loretta Lau1, Bernice Krafchik, Monika M Trebo
1Division of Haematology/Oncology, Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Infants with skin Langerhans cell histiocytosis (LCH) may progress to multi-system disease. Close monitoring is crucial as isolated cutaneous LCH is not always benign.
Area of Science:
- Pediatric Dermatology
- Hematology-Oncology
- Immunology
Background:
- Langerhans cell histiocytosis (LCH) in infants can present with skin involvement.
- Understanding the progression of cutaneous LCH to multi-system disease is critical for infant outcomes.
Purpose of the Study:
- To evaluate the clinical course and outcomes of infants with skin LCH.
- To determine the incidence of progression from isolated skin LCH to multi-system LCH.
Main Methods:
- Retrospective review of 22 infants diagnosed with LCH before 12 months of age.
- Analysis of disease progression, organ involvement, and mortality in patients with cutaneous and multi-system LCH.
Main Results:
- 40% of infants with isolated skin LCH progressed to multi-system disease.
- 79% of multi-system LCH patients had risk organ involvement, with a 50% mortality rate.
- 50% of multi-system LCH cases had a preceding history of skin eruption.
Conclusions:
- Isolated cutaneous LCH in infants requires careful monitoring due to potential progression.
- The diagnosis of self-healing cutaneous LCH should be made retrospectively.
- Non-invasive follow-up is recommended to detect disease progression and long-term complications.
Background:
To evaluate the clinical course and outcome of infants with Langerhans cell histiocytosis (LCH) involving skin and to estimate the incidence of progression to multi-system (M-S) disease in those with isolated skin involvement.
Methods:
A retrospective review was conducted on 22 LCH patients who were younger than 12 months at the onset of their skin eruption.
Results:
Twelve patients had isolated skin involvement at diagnosis and 10 were evaluable for progression. Four of the 10 (40%) evaluable patients progressed to multi-system (M-S) disease. Of the 10 patients with M-S disease at diagnosis, 5 had a history of a preceding skin eruption 2 to 13 months prior to diagnosis. Eleven of the 14 (79%) patients with M-S disease had risk organ involvement. The mortality rate of M-S disease was 50%.
Conclusions:
It is important for primary caregivers to recognize that isolated cutaneous LCH in infants is not always a benign disorder. The diagnosis of self-healing cutaneous LCH should only be made in retrospect. Careful, albeit non-invasive, follow-up is recommended to monitor for disease progression and development of long-term complications.
