A case report of orbital Langerhans cell histiocytosis presenting as a orbital cellulitis

Arch Soc Esp Oftalmol. 2018 Oct;93(10):503-506. doi: 10.1016/j.oftal.2018.03.003. Epub 2018 Apr 8.
[Article in En, Spanish]

Abstract

Clinical case: A 10-year-old girl was seen with a 3-week history of right upper lid swelling and with no other symptoms or fever. There was no recent history of sinusitis, trauma, or previous infection involving the periorbital area, or response to oral antibiotic treatment. Orbital computed tomography showed a lesion involving the upper margin of the orbit, and bone destruction at the orbital roof. Biopsy performed revealed the presence of Langerhans cell Histiocytosis. The lesion was surgically debulked and corticosteroids were used intra-operatively. The lesion responded to treatment.

Discussion: The orbital involvement of Langerhans cell histiocytosis, despite its low incidence, should be considered in the examination of acute peri-orbital swelling. It usually presents as an osteolytic lesion, and it is confirmed with a histological examination and immunohistochemical techniques for CD1a and S100. An interdisciplinary approach is recommended to rule out multifocal or multisystemic diseases, as well as to develop an appropriate treatment strategy.

Keywords: Celulitis orbitaria; Eosinophilic granuloma; Granuloma eosinofílico; Histiocitosis de células de Langerhans; Langerhans cell histiocytosis; Masa orbitaria; Orbital cellulitis; Orbital mass.

Publication types

  • Case Reports

MeSH terms

  • Antigens, CD1 / analysis
  • Biomarkers
  • Child
  • Cytoreduction Surgical Procedures
  • Female
  • Histiocytosis, Langerhans-Cell / complications
  • Histiocytosis, Langerhans-Cell / diagnosis*
  • Histiocytosis, Langerhans-Cell / diagnostic imaging
  • Histiocytosis, Langerhans-Cell / therapy
  • Humans
  • Langerhans Cells / chemistry
  • Orbital Cellulitis / diagnostic imaging
  • Orbital Cellulitis / drug therapy
  • Orbital Cellulitis / etiology*
  • Orbital Cellulitis / surgery
  • Osteolysis / etiology
  • S100 Proteins / analysis
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Triamcinolone / therapeutic use

Substances

  • Antigens, CD1
  • Biomarkers
  • CD1a antigen
  • S100 Proteins
  • Triamcinolone