BULLOUS PEMPHIGOID AND EPIDERMOLYSIS-BULLOSA ACQUISITA - PRESENTATION, PROGNOSIS, AND IMMUNOPATHOLOGY IN 11 CHILDREN

The immunobullous diseases bullous pemphigoid (BP) and epidermolysis b ullosa acquisita (EBA) are very rare in childhood, Although case studi es have been detailed, there are no reports of a large series of patie nts documenting the effectiveness of treatment and longterm prognosis. We report the clinical presentation, immunopathologic features, disea se course, and long-term prognosis of BP and EBA in a series of 11 chi ldren, The initial diagnoses based on clinical features were BP (5), E BA (3), and chronic bullous disease of childhood (CBDC) (3), These wer e subsequently revised from BP to EBA (2), CBDC to BP (2), and CBDC to BP or EBA (1) following the results of direct and indirect immunofluo rescence and immunoblotting. Analysis of IgG subclasses in eight cases showed that the predominant subclasses were IgG1 (8) and IgG4 (8), Th e clinical features appeared to be highly variable, and in patients pr esenting with inflammatory blistering, laboratory studies were require d in order to differentiate between BP and EBA. All patients improved on treatment with corticosteroids and/or sulfones, although treatment regimens showed wide variation, Their diseases tended to remit within 2 years, and their long-term prognosis was good.