A 4 year old child presented with a painful eruption affecting the flexures. Patient was hospitalised and O/E, there was erythema with peeling and blistering of the skin around the lips, eyelid, groin and natal cleft. A few small flaccid blisters were present at the margins of affected areas. The skin of the trunk showed faint erythema. Histological features were not conclusive. Immunofluorescence studies were awaited. What protein family is most likely to be affected by this disease?
Dx: Blistering Disorder- Linear IgA Disease MC protein involvement BPAg2 > BPAg1 Characteristic C/F: String of pearl appearance/cluster of jewel appearance Linear IgA diseaseIt is chronic, acquired sub-epidermal disease of children & adults with cutaneous & mucosal involvement. Two clinical syndromes seen1. Chronic bullous disease of childhood2. Adult linear IgA disease Auto antibodies are IgA (usually IgA1) Chronic Bullous disease of childhood. Linear IgA disease of Adults
Desmocollins
Desmogleins
BPAg1
BPAg2
Blistering disorders