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Langerhans Cell Histiocytosis Presenting in the Neonatal Period
A Retrospective Case Series
Sarah L. Stein, MD;
Amy S. Paller, MD;
Paul R. Haut, MD;
Anthony J. Mancini, MD
Arch Pediatr Adolesc Med. 2001;155:778-783.
Objectives To describe the morphologic characteristics of skin lesions, extent
of extracutaneous disease, and outcomes in patients with neonatal presentation
of Langerhans cell histiocytosis (LCH), and to examine clinical predictors
of disease prognosis.
Design Retrospective validation cohort study. Maximum duration of follow-up
was 10 years.
Setting A tertiary care children's hospital in Chicago, Ill.
Patients Nineteen children with cutaneous findings in the first 4 weeks of life
and subsequently diagnosed with LCH based on compatible tissue histologic
analysis, confirmed by electron microscopy and/or immunohistochemical analysis.
Main Outcome Measure Cutaneous lesion morphologic characteristics, extracutaneous manifestations,
treatments, and outcomes were tabulated and compared.
Results The most common initial skin lesion was erythematous, often crusted,
vesiculopustules. Skin lesion morphologic traits did not correlate with extent
of extracutaneous disease. One third of patients had disease limited to the
skin and/or mucous membranes. All of these patients are alive and well, and
1 has developed diabetes insipidus. Twelve of the 19 patients had multisystem
disease, and 2 died of disease. The results of a multiorgan workup performed
at the time of diagnosis were predictive of which patients in this cohort
manifested multisystem disease. The overall incidence of diabetes insipidus
was 21%.
Conclusions Vesiculopustular lesions are common in congenital/neonatal LCH, but
the morphologic characteristics of lesions are not helpful in predicting the
extent of disease. A multiorgan evaluation at the time of diagnosis may be
predictive of the probability of multisystem involvement with LCH.
From the Departments of Dermatology (Drs Stein, Paller, and Mancini)
and Pediatrics (Drs Paller, Haut, and Mancini), Northwestern University Medical
School, and the Divisions of Dermatology (Drs Stein, Paller, and Mancini)
and Hematology/Oncology (Dr Haut), Children's Memorial Hospital, Chicago,
Ill. Dr Stein is now with the Division of Dermatology, University of Chicago.
Corresponding author and reprints: Anthony J. Mancini, MD, 2300 Children's
Plaza, No. 107, Chicago, IL 60614 (e-mail: amancini{at}northwestern.edu).
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