Faculty Collaboration Database

The natural history of skin-limited Langerhans cell histiocytosis: a single-institution experience. J Pediatr Hematol Oncol 2014 Nov;36(8):613-6

Date

08/30/2014

Pubmed ID

25171449

DOI

10.1097/MPH.0000000000000248

Scopus ID

2-s2.0-84918567015 (requires institutional sign-in at Scopus site)   20 Citations

Abstract

INTRODUCTION: Prior reports of Langerhans cell histiocytosis (LCH) suggest that isolated skin involvement is rare and often progresses to systemic disease. More rapid access to pediatric subspecialty care has likely led to more frequent representation of this condition. The purpose of this study is to characterize the natural history of skin-limited LCH in an era of increased access to pediatric subspecialty care.

MATERIALS AND METHODS: A retrospective chart review was performed on all patients newly diagnosed with LCH between 2001 and 2012 at the Children's Hospital of Wisconsin. Extensive review of laboratory, physical examination, and imaging reports was performed and data collected for patients with biopsy-proven skin LCH.

RESULTS: Sixteen individuals with skin-limited LCH were identified. The median age at onset of skin eruption was birth (range, birth to 6 mo), and median duration of follow-up was 19.5 months (range, 2 wk to 10 y) from diagnosis. One patient (6%) developed pituitary disease and 1 patient (6%) had refractory skin involvement. All others experienced complete resolution. For patients without progressive or refractory disease, resolution of skin findings occurred within 7 months from onset.

DISCUSSION: Progression of skin-limited to multisystem LCH likely may be less frequent than previously described.

Author List

Ehrhardt MJ, Humphrey SR, Kelly ME, Chiu YE, Galbraith SS

Authors

Yvonne E. Chiu MD Vice Chair, Professor in the Dermatology department at Medical College of Wisconsin
Stephen R. Humphrey MD Associate Professor in the Dermatology department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Biopsy
Child
Child, Preschool
Disease Progression
Female
Histiocytosis, Langerhans-Cell
Humans
Infant
Infant, Newborn
Male
Prognosis
Remission, Spontaneous
Retrospective Studies
Skin Diseases