Acquired autoimmune bullous diseases of childhood: Analysis of 22 cases

Acquired autoimmune bullous diseases in children are a relatively rare group of conditions, with overlapping clinical features difficult to differentiate clinically. Hardly any previous studies of acquired autoimmune bullous diseases in children had been reported in Sri Lanka. 22 cases of acquired autoimmune bullous were studied at the skin clinic of Lady Ridgeway Hospital for children over a period of 3 years from May 2004 to May 2007. Based on the clinical presentation, inclusion and exclusion criteria, 22 cases were included in the study. With regards to investigations and treatment no alterations were made to the existing protocols of the unit. Investigations included a skin biopsy and, whenever possible, direct immunofluorescence and relevant haematological investigations. Of the 22, 20 were diagnosed as chronic bullous disease of childhood. and rest as bullous pemphigoid. With regards to diagnosis, there was excellent clinico-pathological correlation. Most affected children were below 5 years of age, and a male preponderance was noted. Significant hypo-albuminaemia was noted in a few of the severely affected. Haemolysis was the most significant side effect of dapsone monotherapy. The combination of dapsone and prednisolone given to severely affected while reducing haemolysis seems to shorten the duration of therapy. No serious side effects of dapsone were noted.