DR. MAYUR MOREKER

Abstract

A 12-year-old boy presented with recurrent drooping of left eye lid on tapering oral steroids. OS showed no vitreous inflammation, mild disc hyperaemia with pocket of subretinal fluid between disc and fovea with prompting a suspicion of “Posterior Scleritis” confirmed by a USG Bscan and OCT Angiography / Macula. His ESR was 19 mm; CRP was Negative. Serum ACE, ANCA – C and P, Serum TSH, Anti-thyroid Antibodies, Serum Uric Acid, Mantoux Test, Serum IgG4, ANA (By IF) were inconclusive. He was started on oral Methotrexate and oral steroids were attempted to be tapered over 6 months.

A relapse at 1.25 mg/day with additional lateral rectus swelling (Orbital Myositis); prompted a shift to Oral Azathioprine 100 mg/day to achieve control of inflammation and cessation of oral steroids in 3 months. Meanwhile, he suffered from Hidradenitis suppurativa (HS); and a diagnosis of PAPASH syndrome was made with a rare clinical association of PAPASH with scleritis.