Adult-onset asthma associated with periocular xanthogranuloma: new diagnostic and therapeutic approaches in a very rare systemic disease

Ophthalmic Plast Reconstr Surg. 2013 Mar-Apr;29(2):104-8. doi: 10.1097/IOP.0b013e31827ae96a.

Abstract

Purpose: To report some novel findings concerning the systemic manifestations and treatment of adult-onset asthma and periocular xanthogranuloma, a rare type of non-Langerhans histiocytosis that can lead to important visual dysfunction.

Methods: A retrospective case series of 2 patients was evaluated for orbital and systemic manifestations using fluorodeoxyglucose positron emission tomography/CT and/or orbital MRI. Histological specimens were reviewed in all patients. Oral prednisone was initiated at 1 mg/kg daily and gradually tapered to a minimum effective dose. Efficacy was assessed on the basis of an objective observation of decreased swelling.

Results: One patient displayed original uptake foci involving intrathoracic lymphadenopathies, the trajectory of the paraumbilical vein and perirectal fat. Low-dose prednisone was able to induce a durable response in the authors' patients.

Conclusions: Fluorodeoxyglucose positron emission tomography/CT may be useful for the diagnostic workup and follow-up assessment of patients with adult-onset asthma and periocular xanthogranuloma. Oral corticosteroids can be used successfully as first-line treatment in such patients.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Asthma / diagnosis*
  • Asthma / drug therapy
  • Eyelid Diseases / diagnosis*
  • Eyelid Diseases / drug therapy
  • Fluorodeoxyglucose F18
  • Glucocorticoids / therapeutic use
  • Granuloma / diagnosis*
  • Granuloma / drug therapy
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Orbital Diseases / diagnosis*
  • Orbital Diseases / drug therapy
  • Positron-Emission Tomography
  • Prednisolone / therapeutic use
  • Radiopharmaceuticals
  • Rare Diseases
  • Retrospective Studies
  • Tomography, X-Ray Computed
  • Xanthomatosis / diagnosis*
  • Xanthomatosis / drug therapy

Substances

  • Glucocorticoids
  • Radiopharmaceuticals
  • Fluorodeoxyglucose F18
  • Prednisolone