Figure 16.1
Tense blisters scattered over lower limb
What is the diagnosis?
1.
Bullous Impetigo
2.
Bullous insect bites
3.
Chronic Bullous disease of Childhood (CBDC)
4.
Childhood bullous pemphigoid
5.
Erythema multiforme
Subsequently Biopsy and IMF were performed. Biopsy revealed a subepidermal blister with a neutrophil rich infiltrate and IMF showed linear deposits of IgA at the DEJ.
The child was diagnosed as CBDC and therapy with Dapsone started at 2 mg/kg. A Blood count was done to check the haemoglobin prior to initiating therapy and Glucose-6-phosphate-dehydrogenase deficiency was excluded. Over the next few months on Dapsone, lesions healed and fewer blisters erupted. After 6 months of therapy, no new lesions occurred and the patient’s Dapsone was stopped.
Chronic Bullous Disease of Childhood
Chronic bullous disease of childhood (CBDC) is an autoimmune blistering disease seen in children. It is self limiting in its course. It begins at least by age 2–3 years with average age of onset is 5 years. CBDC is a childhood variant of linear IgA disease, where circulating IgA autoantibodies against the basement membrane are the cause [1–2]. The immunoreactions IgA antibodies are found in three areas within the basement membrane zone, i.e. the lamina Lucida, at and below the lamina Lucida and above and below the lamina Lucida and therefore multiple targets against BP 230, BP180, and Type VII collagens [1–2].
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