Publication: Atypical Conjunctival Lesion as the Initial Presentation of Granulomatosis with Polyangiitis in an Adolescent Male
0
0
Issued Date
2022
Resource Type
Language
eng
File Type
application/pdf
ISSN
2773740
Rights Holder(s)
Scopus
Bibliographic Citation
International Journal of GEOMATE. Vol 23, No.97 (2022), p.106-114
Suggested Citation
Sripawadkul W., Zein M., Galor A., Rivas-Chacon R., Khzam R.A., Dubovy S.R., Karp C.L. Atypical Conjunctival Lesion as the Initial Presentation of Granulomatosis with Polyangiitis in an Adolescent Male. International Journal of GEOMATE. Vol 23, No.97 (2022), p.106-114. doi:10.1097/ICO.0000000000003015 Retrieved from: https://hdl.handle.net/20.500.14740/10338
Abstract
Purpose:The purpose of this study was to report the first case of a conjunctival granulomatous lesion as the presenting sign of granulomatous polyangiitis (GPA) in a pediatric patient.Methods:This study is a case report.Results:A 14-year-old Hispanic boy presented with a conjunctival lesion on the inferior bulbar conjunctiva of the right eye associated with diffuse conjunctival injection. The mass progressively grew and became painful over the course of 6 weeks. No retinal or orbital abnormalities were noted on examination. The lesion was excised, and histopathological analysis was consistent with granulomatous inflammation. The lesion recurred after 15 months, and a second excisional biopsy was performed. The lesion again slowly recurred, and on presentation to our clinic, an elevated lesion in the inferior limbal/bulbar conjunctiva of the right eye was noted from 4 to 8 o'clock with accompanying forniceal shortening. Five months after the second excision, the patient developed flu-like symptoms with polyarthralgia. A full diagnostic workup revealed multiple pulmonary nodules on chest imaging, proteinuria on urinalysis, and a positive c-antineutrophil cytoplasmic antibody on serological studies. Based on these findings, the patient underwent a kidney biopsy which showed pauci-immune crescentic glomerulonephritis, consistent with a diagnosis of GPA. The patient achieved disease remission with rituximab. Despite treatment, the conjunctival lesion did not regress and remained unchanged in size for 3 years with periodic episodes of inflammation.Conclusions:This is the first documented case of a conjunctival mass as the initial presenting feature of pediatric GPA. The presence of granulomatous inflammation on histopathology and recurrences after excision should raise suspicion for GPA in children and adults. © 2022 Lippincott Williams and Wilkins. All rights reserved.