Ulcerative granuloma of the eyelid as the initial manifestation of granulomatosis with polyangiitis (Wegener's granulomatosis): A case report

Orbit. 2017 Aug;36(4):243-246. doi: 10.1080/01676830.2017.1287740. Epub 2017 Mar 17.

Abstract

A 56-year-old-man presented a 2-month history of chalazion in the eyelids without response to treatment and with an inconclusive biopsy. Laboratory results confirmed the presence of Enterobacter cloacae and Streptococcus gordonii infection. Despite appropriate intravenous antibiotic treatment, clinical worsening was observed. Radical surgical excision and total tarsorrhaphy were performed. Following treatment, the patient was asymptomatic for 6 weeks until he developed acute renal failure, generalised arthralgia, acute hypertensive anterior uveitis, and dacryoadenitis. Lacrimal gland biopsy revealed a perivascular granulomatous inflammation with necrotic foci. Renal biopsy showed a necrotizing extracapillary glomerulonephritis. Blood tests showed elevated levels of C-reactive protein, positive rheumatoid factor, and proteinase 3-anti-neutrophil cytoplasmic antibody. The patients was therefore diagnosed with granulomatosis with polyangiitis and treated with cyclophosphamide and corticosteroids, with good systemic and orbital response. Surgical reconstruction of the eyelid was subsequently performed. This case describes, for the first time in the literature, an eyelid granuloma as the presenting sign in GPA, highlighting the importance of differential diagnosis of eyelid lesions with unusual characteristics.

Keywords: Dacryoadenitis; granulomatosis with polyangiitis; ulcerative granuloma.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / blood
  • C-Reactive Protein / metabolism
  • Cyclophosphamide / therapeutic use
  • Diagnosis, Differential
  • Drug Therapy, Combination
  • Eyelid Diseases / diagnosis*
  • Eyelid Diseases / drug therapy
  • Glucocorticoids / therapeutic use
  • Granuloma / diagnosis*
  • Granuloma / drug therapy
  • Granulomatosis with Polyangiitis / diagnosis*
  • Granulomatosis with Polyangiitis / drug therapy
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Myeloblastin / blood
  • Tomography, X-Ray Computed
  • Ulcer / diagnosis*
  • Ulcer / drug therapy

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Glucocorticoids
  • Immunosuppressive Agents
  • Cyclophosphamide
  • C-Reactive Protein
  • Myeloblastin
  • Methylprednisolone