ARTICLE Langerhans Cell 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

Objectives: To describe the morphologic characteris- Results: The most common initial skin lesion was ery- tics of skin lesions, extent of extracutaneous disease, and thematous, often crusted, vesiculopustules. Skin lesion outcomes in patients with neonatal presentation of morphologic traits did not correlate with extent of ex- Langerhans cell histiocytosis (LCH), and to examine tracutaneous disease. One third of patients had disease clinical predictors of disease prognosis. limited to the skin and/or mucous membranes. All of these patients are alive and well, and 1 has developed diabetes Design: Retrospective validation cohort study. Maxi- insipidus. Twelve of the 19 patients had multisystem dis- mum duration of follow-up was 10 years. ease, and 2 died of disease. The results of a multiorgan workup performed at the time of diagnosis were predic- Setting: A tertiary care children’s hospital in Chicago, tive of which patients in this cohort manifested multi- Ill. system disease. The overall incidence of diabetes insipi- dus was 21%. Patients: Nineteen children with cutaneous findings in the first 4 weeks of life and subsequently diagnosed with Conclusions: Vesiculopustular lesions are common in LCH based on compatible tissue histologic analysis, con- congenital/neonatal LCH, but the morphologic charac- firmed by electron microscopy and/or immunohisto- teristics of lesions are not helpful in predicting the ex- chemical analysis. tent of disease. A multiorgan evaluation at the time of diagnosis may be predictive of the probability of multi- Main Outcome Measure: Cutaneous lesion morpho- system involvement with LCH. logic characteristics, extracutaneous manifestations, treat- ments, and outcomes were tabulated and compared. Arch Pediatr Adolesc Med. 2001;155:778-783

HE HISTIOCYTOSES are a group Patients with LCH demonstrate a va- of disorders that encom- riety of clinical presentations and pos- passes a wide range of pri- sible outcomes. The term LCH and the his- mary and secondary, soli- torical term histiocytosis X encompass 3 tary and multiple, and classic clinical entities, which are now benignT and malignant conditions. They are considered to be variations of the same unified by their common cell of origin, the disease: (1) (lo- histiocyte. The clinical findings associated calized lesions in bone); (2) Hand- with these conditions depend on the ex- Schu¨ ller-Christian disease (multiple or- From the Departments of tent of organ systems involved. Several clas- gan involvement with the classic triad of Dermatology (Drs Stein, Paller, sification schemes have been proposed to skull defects, diabetes insipidus [DI], and and Mancini) and Pediatrics better delineate the specific conditions and exophthalmos); and (3) Letterer-Siwe dis- (Drs Paller, Haut, and to decrease confusion stemming from the ease (visceral lesions involving multiple Mancini), Northwestern historical use of multiple eponyms. The organs).1 A fourth clinical entity termed University Medical School, and most familiar classification includes Langer- congenital self-healing reticulohistiocyto- the Divisions of Dermatology hans cell histiocytosis(LCH), non-Langerhans sis (Hashimoto Pritzker variant) has been (Drs Stein, Paller, and cell histiocytoses, and malignant histiocytic described in which skin lesions are pres- Mancini) and disorders. The presence or absence of the pa- ent at birth, accompanied in rare cases by Hematology/Oncology thognomonic Langerhans cell organelle, the systemic findings, and with complete spon- (Dr Haut), Children’s Memorial 2,3 Hospital, Chicago, Ill. Dr Stein Birbeck granule, within the pathologic his- taneous involution within 2 to 3 months. is now with the Division tiocyte, and the findings on immunohisto- Pa