title: Langerhans cell histiocytosis with seborrheic eczema of the scalp and extensive calvarial involvement.
abstract: BACKGROUND
  Langerhans cell histiocytosis (LCH) is a multisystem disorder of unknown etiology and characterized by accumulation of histiocytes in various tissues.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  LCH may cause progressive calvarial defects. If seborrheic eczemas are concurrent, they may suggest prompt histological verification and treatments be initiated.
authors.affiliation: Department of Neurological Surgery, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba, 279-0021, Japan. shotaro@juntendo-urayasu.jp.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Tsutsumi
authors.affiliation: Department of Neurological Surgery, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba, 279-0021, Japan.
authors.forename: Shintaro
authors.initials: S
authors.lastname: Nakajima
authors.affiliation: Department of Pediatrics, Juntendo University Urayasu Hospital, Urayasu, Chiba, Japan.
authors.forename: Hisayuki
authors.initials: H
authors.lastname: Oda
authors.affiliation: Department of Neurological Surgery, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba, 279-0021, Japan.
authors.forename: Yukimasa
authors.initials: Y
authors.lastname: Yasumoto
mesh_terms: D002675:Child, Preschool; D019465:Craniofacial Abnormalities / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D004485:Eczema / Q000150:complications* / Q000000981:diagnostic imaging; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D021621:Imaging, Three-Dimensional; D008279:Magnetic Resonance Imaging; D009418:S100 Proteins / Q000378:metabolism; D012535:Scalp / Q000000981:diagnostic imaging / Q000473:pathology*; D014057:Tomography, X-Ray Computed
chemical_list: D009418:S100 Proteins
keywords: Extensive bone defects; Langerhans cell histiocytosis; Seborrheic eczema
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