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Langerhans cell histiocytosis with seborrheic eczema of the scalp and extensive calvarial involvement

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Abstract

Introduction

Langerhans cell histiocytosis (LCH) is a multisystem disorder of unknown etiology and characterized by accumulation of histiocytes in various tissues.

Case report

A 3-year-old, previously healthy girl presented with progressive flattening of the parietal convexity for 6 months and seborrheic eczema of the scalp. At presentation, the patient showed no neurological deficit. The eczemas were extensively distributed over the scalp, but not found in any other site of the body. Blood examination revealed a marked increase in soluble interleukin-2 receptor levels. Neuroimages revealed multiple calvarial defects that were replaced by well-demarcated, enhancing extracerebral masses. A biopsy surgery confirmed the diagnosis as LCH.

Conclusion

LCH may cause progressive calvarial defects. If seborrheic eczemas are concurrent, they may suggest prompt histological verification and treatments be initiated.

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Acknowledgments

This work was not supported by a grant.

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Authors and Affiliations

  1. Department of Neurological Surgery, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba, 279-0021, Japan

    Satoshi Tsutsumi, Shintaro Nakajima & Yukimasa Yasumoto

  2. Department of Pediatrics, Juntendo University Urayasu Hospital, Urayasu, Chiba, Japan

    Hisayuki Oda

Authors
  1. Satoshi Tsutsumi
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  2. Shintaro Nakajima
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  3. Hisayuki Oda
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  4. Yukimasa Yasumoto
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Corresponding author

Correspondence to Satoshi Tsutsumi.

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Conflict of interest

The authors declare no competing interests concerning either the materials and methods, or the findings presented in this study.

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Cite this article

Tsutsumi, S., Nakajima, S., Oda, H. et al. Langerhans cell histiocytosis with seborrheic eczema of the scalp and extensive calvarial involvement. Childs Nerv Syst 32, 1337–1341 (2016). https://doi.org/10.1007/s00381-016-3026-1

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  • DOI: https://doi.org/10.1007/s00381-016-3026-1

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