Juvenile blistering diseases: the problems of diagnosis and treatment

Abstract

Correct clinical diagnosis in cases of chronic, relapsing, non-hereditary, blistering diseases in childhood could not be made without the aid of histopathology and immunofluorescence, since the morphology and the distribution of the lesions of bullous pemphigoid (BP), linear IgA bullous dermatosis (LAD) and dermatitis herpetiformis (DH) may be similar. Histopathology was helpful in about half of the cases. The results of immunopathology were very useful for the final diagnosis. Of twenty-one cases of juvenile blistering diseases, two cases which showed IgG on direct test with circulating antibodies were BP; three cases with deposition of IgG but without circulating antibodies were probably BP; three cases were either BP or LAD (IgG and IgA on direct test without circulating antibodies); nine cases were definite LAD (linear IgA only); one case which showed granular IgA in the dermal papillae and linear IgA was DH; and the last three cases were probably LAD and BP with non-immunoreactant deposits. Regardless of the diagnosis, dapsone and co-trimoxazole controlled eight cases and could be discontinued without relapse, while the other six cases were maintained on dapsone therapy alone. Prednisolone, when added in cases of poor response to either dapsone or co-trimoxazole, caused remission and was discontinued in three out of the five cases. Two cases were lost to follow up.