Wiskott‐Aldrich syndrome: A case with scleritis

• Published in: Acta ophthalmologica (Oxford, England) Vol. 87; no. s244
• Main Authors: KARAMPATAKIS, V, HATZISTILIANOU, M, KORDALI, P, BISMPAS, I
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01-09-2009
• ISSN: 1755-375X; 1755-3768
• DOI: 10.1111/j.1755-3768.2009.413.x

Purpose To present a case of scleritis in a patient with Wiskott‐Aldrich primary immunodeficiency.
Methods An 18 year old male presented with red left eye. The patient received immunoglobulins for his immunodeficiency regularly. Dexamethasone eye drops were prescribed. In two days the redness increased and the eye became painful mainly nasally with a slight diminution of visual acuity(VA).
Results Aqueous flare and cells were detected. Intraocular pressure (IOP) was 12 mmHg. Steroid eye drops (dexamethasone) in frequent instillation and diclofenac eye drops were administered. Acyclovir (per os 800mgrx5) was added. Seven days later the redness increased and gradually the eye became more painful (nasally). Prednisolone 2, 5 % (1x1 at night) was added and also naproxen tablets (250mgrx3). Within 2 days the aqueous flare and cells ameliorated. Four days later a marginal corneal infiltration appeared nasally. Within the next week an increase of the lateral scleral involvement appeared (the nasal part was obviously better) and this lateral scleral area became painful. The fundus examination revealed chorioretinal involvement corresponding to the lateral scleral painful area. The visual acuity and the IOP remained normal. The patient was instructed to continue his therapy. He also received gamma globulins regularly (100mg/Kg/week). Only after the lateral deterioration of scleritis an additional dose was added in the middle of the week. The laboratory findings were indicative of inflammation.
Conclusion The topical treatment with dexamethasone, diclofenac and prednisolone in conjuction with acyclovir and naproxen per os was proved adequate treatment for this particular patient who also received gammaglobulins.