Due to their rarity, the chrotiic acquired bullous diseases of childhood have not yet beeti studied in detail. Constant changes in terminology and classification have so far achieved little apart from confusing clinicians. We have attempted, therefore, to interpret the literature, to define the diseases in question and have included some case reports of patients seen recently at St John's Hospital for Diseases of the Skin.
The pemphigus groupWhen pemphigus occurs in children, the disease is chtiically, histologically and immunologically similar to that in adults. Beutner & Chorzelski (1976) reviewed 234 cases of pemphigus and found that the disease began before the age of 10 in four cases and between 11 and 20 years in nine. Eleven of these cases were female, an incidence 5-5 times higher than in other age groups, where the sexes are equally affected. This female preponderance in children with pemphigus has been noted elsewhere (Jordon et al, 1976). It is of interest that a much larger proportion of affected children had pemphigus foliaceus or erythematosus (sis out of thirteen cases) compared with adults (twenty-seven out of 221 cases).
The direct immunofiuorescence of bullous disorders and lupus erythematosus was compared using a liquid fixative and a standard freezing method. Sections were left in fixative for i, 3 or 5 days before processing. The intensity of fiuorescence faded and became absent in some cases where the sections were in fixative for 3 or 5 days. This may on occasion give a false negative result.
Direct immunofluorescence was done on smears taken from 38 cases of bullous dermatoses and oral ulceration. 16 of these had pemphigus. All of the pemphigus cases had positive Tzanck smears and fluorescence of individual acantholytic cells and/or intercellular fluorescence of sheets of cells. Other bullous dermatoses showed no acantholysis or fluorescence.Smears from a series of oral lesions (mainly aphthous ulcers) showed intercellular fluorescence of sheets of cells similar to pemphigus. Therefore smear immunofluorescence cannot be reliably used as a diagnostic test in oral pemphigus.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.