title: Langerhans cell histiocytosis in childhood - review, symptoms in the oral cavity, differential diagnosis and report of two cases.
abstract: BACKGROUND
  Langerhans cell histiocytosis is a rare disease characterized by monoclonal proliferation and migration of special dendritic cells. This disease primarily affects bones, but occurs less frequently in other organ systems or may manifest as a multisystem disease.
  
  
  METHODS
  Extraoral and intraoral symptoms of Langerhans cell histiocytosis are described in a 13-month-old female and a 5-month-old male infant. Dermatitis was found on the scalp, abdomen, flexures and in intertrigineous areas in both patients. The intraoral examination of the 13-month-old infant showed premature eruption of all maxillary deciduous molars, loosening and significant damage of periodontal tissues (gingivitis with bleeding, swelling of palatal mucosa, periodontal pockets) resembling severe periodontitis. In the oral cavity of the 5-month-old predentate infant bilateral swellings of maxillary alveolar mucosa with deep ulcerations were seen. The oral and skin symptoms in both infants were indications for biopsy. Langerhans cell histiocytosis was confirmed histologically and immunohistochemically.
  
  
  CONCLUSIONS
  Oral findings in Langerhans cell histiocytosis may be the only clinical symptom of the disease; therefore the role of dentists in establishing diagnosis is very important.
authors.affiliation: Department of Dentistry (Head of Department: as. Prof. Dr. Antonin Zicha, CSc.), University Hospital and Faculty of Medicine in Pilsen, Charles University in Prague, Alej Svobody 80, 304 60 Pilsen, Czech Republic.
authors.forename: Vlasta
authors.initials: V
authors.lastname: Merglová
authors.affiliation: Department of Oral and Maxillofacial Surgery (Head of Department: Dr. Daniel Hrusak, Ph.D.), University Hospital and Faculty of Medicine in Pilsen, Charles University in Prague, Alej Svobody 80, 304 60 Pilsen, Czech Republic. Electronic address: hrusak@fnplzen.cz.
authors.forename: Daniel
authors.initials: D
authors.lastname: Hrušák
authors.affiliation: Sikl's Department of Pathology (Head of Department: Prof. Dr. Michal Michal), University Hospital and Faculty of Medicine in Pilsen, Charles University in Prague, Dr. Edvarda Benese 13, 305 99 Pilsen, Czech Republic.
authors.forename: Ludmila
authors.initials: L
authors.lastname: Boudová
authors.affiliation: Sikl's Department of Pathology (Head of Department: Prof. Dr. Michal Michal), University Hospital and Faculty of Medicine in Pilsen, Charles University in Prague, Dr. Edvarda Benese 13, 305 99 Pilsen, Czech Republic.
authors.forename: Petr
authors.initials: P
authors.lastname: Mukenšnabl
authors.affiliation: Department of Dentistry (Head of Department: as. Prof. Dr. Antonin Zicha, CSc.), University Hospital and Faculty of Medicine in Pilsen, Charles University in Prague, Alej Svobody 80, 304 60 Pilsen, Czech Republic.
authors.forename: Eva
authors.initials: E
authors.lastname: Valentová
authors.affiliation: Department of Oral and Maxillofacial Surgery (Head of Department: Dr. Daniel Hrusak, Ph.D.), University Hospital and Faculty of Medicine in Pilsen, Charles University in Prague, Alej Svobody 80, 304 60 Pilsen, Czech Republic.
authors.forename: Lubor
authors.initials: L
authors.lastname: Hostička
mesh_terms: D001706:Biopsy; D003872:Dermatitis / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D005884:Gingival Hemorrhage / Q000175:diagnosis; D005891:Gingivitis / Q000175:diagnosis; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D008297:Male; D008963:Molar / Q000503:physiopathology; D009059:Mouth Diseases / Q000175:diagnosis*; D019226:Oral Ulcer / Q000175:diagnosis; D010514:Periodontal Pocket / Q000175:diagnosis; D010518:Periodontitis / Q000175:diagnosis; D012536:Scalp Dermatoses / Q000175:diagnosis; D013280:Stomatitis / Q000175:diagnosis; D014078:Tooth Eruption / Q000502:physiology; D014094:Tooth, Deciduous / Q000503:physiopathology
keywords: Bone lesions; Dermatitis; Granulomatous gingivitis; Infants; Langerhans cell histiocytosis; Periodontitis