Maurice F. Rabb » Uveitis

This 12 year old white female was referred for evaluation of uveitis. Her past medical history was as follows:

At age 7, flu-like illness followed by nephrotic syndrome.
At age 8, hematuria and skin rash.
At age 9, generalized skin rash. Renal biopsy revealed mesangial proliferative glomerulonephritis with granular deposition of Ig C, IgM, C3, C4.
At age 10, asthmatic bronchitis and generalized edema.
At age 11, recurrence of generalized edema, skin rash, and fever associated wtih loss of all body hair, butterfly maculopapillar rash and proteinuria.

In February 1985, at age 12, developed red eyes and photophobia. Visual acuity in the right eye was 20/25, and in the left eye was 20/40. The optic nerve was swollen. There were anterior uveitis, scattered yellow retinal lesions in the periphery, and vitreous cells.

In March, 1985, the patient developed subretinal elevated yellowish parafoveal lesions in the left eye, and visual acuity was recorded as 20/80.

In June 1985, the visual acuity was 20/25 in both eyes, correctable to 20/20 and J-1. There were cells in the anterior chamber and vitreous. Funduscopic examination revealed evidence of the pseudo-POHS syndrome. There was angiographic evidence of choroidal neovascularization in the papillomacular bundle of both eyes.

IMPRESSION:
Pseudo-POHS syndrome occurring in a patient with nephrotic syndrome and chronic glomerulonephritis.