Chronic bullous dermatosis of childhood, also termed linear IgA dermatosis, is the most common acquired autoimmune blistering disorder of childhood and is characterized by itchy, urticated papules and plaques as well as polycyclic lesions (merged circles) with blisters at the edge, located on normal looking skin around the mouth and perineum in young children. In children over 7 years, other parts of the body may rather be affected.
Q: What tissue is targeted in chronic bullous dermatosis of childhood?
A: In chronic bullous dermatosis of childhood, there is an autoimmune attack on structural proteins, usually proteolytic fragments of collagen XVII, which renders the dermal-epidermal junction prone to blistering.
The dermal-epidermal junction is where the surface skin layer, or epidermis, meets the lower layer, or dermis. Diagnosis is confirmed by characteristic histology and direct immunofluorescence showing linear IgA (immunoglobulin A antibody) staining of the basement membrane zone.1
The incidence of chronic bullous disease of childhood is rare. Age of onset is typically before 5 years of age and is seen in all ethnic groups.
What Is Chronic Bullous Dermatosis Of Childhood In Celiac Disease and/or Gluten Sensitivity?
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Mintz EM, Morel KD.Clinical features, diagnosis, and pathogenesis of chronic bullous disease of childhood. Dermatol Clin. 2011 Jul;29(3):459-62, ix. doi: 10.1016/j.det.2011.03.022. ↩