A 2 YEARS OLD GIRL WITH LINEAR IgA DERMATOSIS OF CHILDHOOD (CHRONIC BULLOUS DERMATOSIS OF CHILDHOOD) A CASE REPORT
Debby Andina,Ganung Harsono,Diah Lintang Kawuryan
Departement of Child Health Faculty of Medicine Sebelas Maret University
Background : Linear IgA bullous dermatosis (LABD) is a rare autoimmune skin disorder with the blisters formed in the skin and mucous membranes. LABD incidence is estimated at 1 per 1 million people annually. It is the most common autoimmune bullous disorder in children. The onset is between 1 and 11 years old. Direct immunofluorescence of a skin biopsy demonstrates the line of IgA antibodies just below the epidermis.
Case Presentation Summary : A 2 years old girl admitted to Dr. Moewardi General Hospital with blister all over her body. Physical examination revealed blisters filled with fluid and bullous eruption in her body. Her facial skin thickened and her scalp is scally. The mucous tissue biopsy demonstrated subepidermal blister containing inflammatory fibrin or neutrophil cells and eosinophil spongiosis. Indirect immunofluorescence revealed binding of IgA to the epidermal side of the lamina lucida which established the diagnosis of linear IgA bullous dermatosis. This patient was treated with azitromycin with the dose of 50 mg daily for about three weeks and metylprednisolone with the dose of 10 mg daily for three weeks. Monthly visit was planned for routine monitoring.
Learning Points/Discussion : As this rare disease can present in such a heterogenous and polymorphic fashion, performing a biopsy is crucial to achieve an accurate diagnosis and subsequent treatment. Majority of patients respond to dapsone, sulfapiridine or systemic steroids in cases of widespread disease. However, dapsone is not available in our hospital thus we gave azitromycin which was also efficacious. The duration of treatment is individualized ranging from 3 to 21 months.
Keywords: Chronic bullous dermatosis of childhood Linear IgA dermatosis of childhood CBDC Autoimmune skin disorder
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