A Case Of Childhood Iga Bullous Dermatosis With Ocular Involvement: A Diagnostic Challenge

IgA bullous dermatosis of childhood is a rare autoimmune blistering disorder that typically presents with cutaneous manifestations, but may involve ocular structures. This case report highlights diagnostic challenges and treatment strategies of a 3-year-old girl with corneal involvement, initially misdiagnosed with a viral aetiology.

Ophthalmology clinic and multidisciplinary care involving the paediatric team and dermatology team, where the patient was managed and investigated for ocular and dermatologic symptoms.

A 3-year-old girl presented to the eye clinic with a four week history of left eye redness, photophobia, and lid swelling, preceded by a widespread blistering rash. Examination under anaesthesia (EUA) revealed findings suggestive of a shield ulcer, treated conservatively with topical steroids, lubricants, and antibiotics. Two weeks later, the corneal ulcer worsened, prompting the need for a temporary partial tarsorrhaphy. Suspecting a viral aetiology, antiviral therapy was commenced, and topical steroids discontinued.

As her skin condition deteriorated, she was referred to dermatology and autoimmune screening revealed elevated ANCA MPO of 61.6 (reference range: 0-19.9). A second EUA showed worsening corneal ulcers, scarring, and conjunctival inflammation in the left eye with similar appearances beginning in the right eye. An amniotic membrane graft was placed in the left eye and the diagnosis of IgA bullous dermatosis was confirmed by a skin biopsy showing sub-epidermal blisters and linear IgA deposits.

The patient was started on oral steroids, resulting in significant improvement in her skin and ocular condition. Repeat EUA after one week showed early signs of improvement although Haemophilus influenzae was cultured. Another amniotic membrane graft was placed in the left eye and she was started on levofloxacin eye drops. A subsequent EUA showed complete resolution of the epithelial defect. Following stabilization, she was transitioned to dapsone for long-term management.

This case underscores the importance of considering autoimmune conditions in the differential diagnosis of paediatric ocular ulcers, especially when accompanied by cutaneous manifestations. Early recognition and prompt intervention with appropriate immunosuppressive therapy can lead to significant clinical improvement, highlighting the value of a multidisciplinary approach in managing complex cases like this.