Atypical presentation of isolated orbital Langerhans cell histiocytosis.
Antigens, CD1
/ analysis
Biomarkers
/ analysis
Biopsy
Child
Female
Histiocytosis, Langerhans-Cell
/ diagnostic imaging
Humans
Immunohistochemistry
Orbit
/ chemistry
Orbital Diseases
/ diagnostic imaging
Prednisone
/ therapeutic use
S100 Proteins
/ analysis
Treatment Outcome
Vinblastine
/ therapeutic use
Langerhans cell histiocytosis
bone lesions
orbital tumors
pediatric tumors
Journal
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG
ISSN: 2532-179X
Titre abrégé: Sarcoidosis Vasc Diffuse Lung Dis
Pays: Italy
ID NLM: 9610928
Informations de publication
Date de publication:
2019
2019
Historique:
received:
27
08
2018
accepted:
05
11
2018
entrez:
2
6
2020
pubmed:
1
1
2019
medline:
15
7
2020
Statut:
ppublish
Résumé
A 9-year old female presented with one month of waxing and waning upper eyelid swelling. An excisional biopsy via anterior orbitotomy was performed. To describe a patient presenting atypically with symptoms concerning for orbital cellulitis who was diagnosed with Langerhans cell histiocytosis (LCH). Description of case report. We report a case of a 9-year old female with one month of periorbital edema and erythema suspected to be orbital cellulitis. A complete ophthalmological exam, subsequent imaging, and an excisional biopsy revealed the diagnosis of LCH. With a confirmed diagnosis, the patient started chemotherapy indicated by the Histiocyte Society Evaluation and Treatment Guidelines. Langerhans cell histiocytosis (LCH) embodies a spectrum of diseases with the primary pathologic process being the abnormal proliferation of polyclonal Langerhans cells. In children with isolated bony involvement, the most common presenting symptom is pain. Rarely is orbital involvement with associated periorbital edema and erythema the primary presentation.
Sections du résumé
BACKGROUND
BACKGROUND
A 9-year old female presented with one month of waxing and waning upper eyelid swelling. An excisional biopsy via anterior orbitotomy was performed.
OBJECTIVE
OBJECTIVE
To describe a patient presenting atypically with symptoms concerning for orbital cellulitis who was diagnosed with Langerhans cell histiocytosis (LCH).
METHODS
METHODS
Description of case report.
RESULTS
RESULTS
We report a case of a 9-year old female with one month of periorbital edema and erythema suspected to be orbital cellulitis. A complete ophthalmological exam, subsequent imaging, and an excisional biopsy revealed the diagnosis of LCH. With a confirmed diagnosis, the patient started chemotherapy indicated by the Histiocyte Society Evaluation and Treatment Guidelines.
CONCLUSION
CONCLUSIONS
Langerhans cell histiocytosis (LCH) embodies a spectrum of diseases with the primary pathologic process being the abnormal proliferation of polyclonal Langerhans cells. In children with isolated bony involvement, the most common presenting symptom is pain. Rarely is orbital involvement with associated periorbital edema and erythema the primary presentation.
Identifiants
pubmed: 32476950
doi: 10.36141/svdld.v36i2.7639
pii: SVDLD-36-167
pmc: PMC7247102
doi:
Substances chimiques
Antigens, CD1
0
Biomarkers
0
CD1a antigen
0
S100 Proteins
0
Vinblastine
5V9KLZ54CY
Prednisone
VB0R961HZT
Types de publication
Case Reports
Langues
eng
Pagination
167-171Informations de copyright
Copyright: © 2019.
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