Multisystem Langerhans' cell histiocytosis (Hand-Schüller-Christian disease) in an adult: a case report and review of the literature.

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Version: author
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Serval ID
serval:BIB_F917D925F59C
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Multisystem Langerhans' cell histiocytosis (Hand-Schüller-Christian disease) in an adult: a case report and review of the literature.
Journal
European Archives of Oto-rhino-laryngology
Author(s)
Scolozzi P., Lombardi T., Monnier P., Jaques B.
ISSN
0937-4477 (Print)
ISSN-L
0937-4477
Publication state
Published
Issued date
2004
Volume
261
Number
6
Pages
326-330
Language
english
Notes
Publication types: Case Reports ; Journal Article ; Review Status: ppublish
Abstract
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.
Keywords
Adult, Alveolar Bone Loss/radiography, Biopsy, Needle, Diabetes Insipidus, Neurogenic/diagnosis, Diagnosis, Differential, Follow-Up Studies, Histiocytosis, Langerhans-Cell/diagnosis, Humans, Hypopituitarism/diagnosis, Immunohistochemistry, Magnetic Resonance Imaging, Male, Mastoiditis/diagnosis, Middle Aged, Radionuclide Imaging/methods, Severity of Illness Index, Tomography, X-Ray Computed
Pubmed
Web of science
Open Access
Yes
Create date
03/09/2011 20:13
Last modification date
01/10/2019 6:20
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