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Langerhans cell histiocytosis with presentation as orbital disease.

Bhanage AB, Katkar AD, Ghate PS - J Pediatr Neurosci (2015 Apr-Jun)

Bottom Line: Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs.In rare instances, the affection of the orbit is the only and the first symptom.He was treated with salvage therapy for risk patients achieving complete remission.

View Article: PubMed Central - PubMed

Affiliation: Department Neurosurgery, Ruby Hall Clinic, Pune, Maharashtra, India.

ABSTRACT
Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration) with acute inflammation (1-week duration) giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009) but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement) and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission.

No MeSH data available.


Related in: MedlinePlus

HPE showing giant cells and Langerhans cells
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Figure 2: HPE showing giant cells and Langerhans cells

Mentions: On histopathology, sheets of histiocytic cells with indented pale nuclei with nuclear grooves suggestive of LCH were seen. Positive immunohistochemical staining for S-100 confirmed the diagnosis [Figure 2].


Langerhans cell histiocytosis with presentation as orbital disease.

Bhanage AB, Katkar AD, Ghate PS - J Pediatr Neurosci (2015 Apr-Jun)

HPE showing giant cells and Langerhans cells
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4489065&req=5

Figure 2: HPE showing giant cells and Langerhans cells
Mentions: On histopathology, sheets of histiocytic cells with indented pale nuclei with nuclear grooves suggestive of LCH were seen. Positive immunohistochemical staining for S-100 confirmed the diagnosis [Figure 2].

Bottom Line: Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs.In rare instances, the affection of the orbit is the only and the first symptom.He was treated with salvage therapy for risk patients achieving complete remission.

View Article: PubMed Central - PubMed

Affiliation: Department Neurosurgery, Ruby Hall Clinic, Pune, Maharashtra, India.

ABSTRACT
Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration) with acute inflammation (1-week duration) giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009) but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement) and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission.

No MeSH data available.


Related in: MedlinePlus