A Case of Linear IgA Bullous Dermatosis of Childhood With Ocular Involvement: A Diagnostic Challenge

PURPOSE: Linear IgA bullous dermatosis of childhood is a rare autoimmune blistering disorder that typically presents with cutaneous manifestations but may involve ocular structures. This case highlights diagnostic challenges and treatment strategies of a 3-year-old girl with corneal involvement, initially misdiagnosed with a viral etiology. METHODS: The patient presented with left-eye redness, photophobia, and eyelid swelling, preceded by a blistering rash. Evaluation included ocular examinations under anesthesia (EUA), autoimmune screening, and skin biopsy with direct immunofluorescence. Pediatric and dermatology teams provided joint care. RESULTS: Initial EUA identified a shield ulcer in the left cornea, managed with topical antibiotics, corticosteroids, and lubricants. Despite this, the ulcer progressed, necessitating partial tarsorrhaphy. Antiviral therapy was initiated when viral keratitis was suspected. As the cutaneous eruption advanced, dermatology review was obtained, and autoimmune testing revealed elevated MPO-ANCA. A repeat EUA demonstrated worsening corneal ulceration with conjunctival inflammation and early contralateral involvement. An amniotic membrane graft was applied. Skin biopsy confirmed linear IgA bullous dermatosis with subepidermal blisters and linear IgA deposits. Systemic corticosteroids led to marked improvement in ocular and cutaneous disease. Follow-up EUA showed early epithelial healing, although Haemophilus influenzae was cultured and treated with topical levofloxacin. A second graft was required, after which EUA confirmed complete resolution of the epithelial defect. The patient was transitioned to dapsone for long-term management. CONCLUSIONS: Autoimmune blistering disease should be considered in children with ocular ulcers, especially when accompanied by cutaneous manifestations. Early biopsy, timely diagnosis, and multidisciplinary management can lead to significant clinical improvement.