Cutaneous Langerhans cell histiocytosis in children under one year

University of Toronto, Toronto, Ontario, Canada
Pediatric Blood & Cancer (Impact Factor: 2.56). 01/2006; 46(1):66-71. DOI: 10.1002/pbc.20479
Source: PubMed

ABSTRACT 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.
A retrospective review was conducted on 22 LCH patients who were younger than 12 months at the onset of their skin eruption.
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%.
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.

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    ABSTRACT: Langerhans cell histiocytosis (LCH) is the most common type of childhood histiocytic disorder with an incidence of 0.2 to 1 per 100,000 children under the age of 15 years (1). The clinical picture of LCH varies from single system (S-S)bone or skin disease to multi-system disease (M-S) (2). We report a neonate with disseminated papulonodular eruption containing mononuclear CD1a and S100 positive histiocytic cells infiltration at epidermis and underlying dermis. The diagnosis of a congenital self healing Langerhans cell histiocytosis (CSHLCH) was made and follow up showed a complete recovery of the eruptions , leaving hypopigmented macules in the sites corresponding to the initial findings.
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