Multisystem Langerhans' cell histiocytosis (Hand-Schüller-Christian disease) in an adult: a case report and review of the literature.
- Scolozzi P Division of Oral and Maxillofacial Surgery, Centre Hospitalier Universitaire Vaudois (CHUV), 1011 Lausanne, Switzerland. scolozzi@hotmail.com
- Lombardi T
- Monnier P
- Jaques B
- 2003-10-11
Published in:
- European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery. - 2004
Adult
Alveolar Bone Loss
Biopsy, Needle
Diabetes Insipidus, Neurogenic
Diagnosis, Differential
Follow-Up Studies
Histiocytosis, Langerhans-Cell
Humans
Hypopituitarism
Immunohistochemistry
Magnetic Resonance Imaging
Male
Mastoiditis
Middle Aged
Radionuclide Imaging
Severity of Illness Index
Tomography, X-Ray Computed
English
Langerhans' cell histiocytosis (LCH) is a rare and enigmatic clonal disorder that affects mainly children. It is characterized by single or multiple granulomatous mass lesions composed of cells with the Langerhans' cell phenotype. Clinical presentation and behavior are heterogeneous and can range from a solitary lytic bone lesion (i.e., eosinophilic granuloma) with a favorable course to a fatal disseminated leukaemia-like form, with a wide spectrum of intermediate clinical presentations between these two extremes. Although LCH typically involves the bone, lesions can be found in almost all organs. We are reporting the case of a multisystem LCH in a 47-year-old patient who presented with a panhypopituitarism and diabetes insipidus, and who, 5 years later, developed mandibular, mastoid and femoral lesions. The final diagnosis of LCH was made on mandibular biopsy.
- Language
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- English
- Open access status
- green
- Identifiers
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- DOI 10.1007/s00405-003-0690-z
- PMID 14551790
- Persistent URL
- https://folia.unifr.ch/global/documents/282866
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