Chronic bullous dermatosis of childhood associated with coeliac disease in a 6-year-old boy.

Sir, Many of the diseases reported in association with coeliac disease (CD) are linked to the histocompatibility antigen HLA-DR3, such as dermatitis herpetiformis (DH) and Type 1 diabetes mellitus (1, 2). DH is characterized by granular IgA deposits in the basement membrane zone. It has recently been shown that epidermal transglutaminase is the autoantigen of DH, as opposed to CD, in which tissue transglutaminase is the main autoantigen (3). DH is rare in coeliac children. Thus, in our Paediatric Clinic we have over the past 25 years diagnosed only one case of DH among 220 consecutive cases of CD. The bullous disease referred to as chronic bullous dermatosis of childhood (CBDC) typically presents as itchy, urticated papules and plaques as well as polycyclic lesions with blisters at the edge, located in the face and perineum (4). CBDC is distinguished from DH, apart from differences in clinical appearance and age at onset, by exhibiting linear deposits of IgA at the basement membrane zone. The same pattern of IgA is seen in linear IgA disease of adults. There is much overlap between these diseases (5). However, according to the literature, CBDC, unlike DH, is not associated with gluten-sensitive enteropathy (6), although this is contradicted by the present case report.