A prospective study of 77 consecutive patients with bullous diseases was done to study the pattern and natural history. Pemphigus was the commonest with 45 patients (59%) followed by pemphigoid with 21 patients (27%). Pemphigoid was more common in Indians than in other ethnic groups and its age of onset was a decade later than pemphigus. Unusual immunofluorescent findings in both diseases are discussed. Six of the 7 patients with dermatitis herpetiformis had linear IgA in the dermo-epidermal junction and the classical papillary IgA deposits were absent. Ultrastructural findings of pemphigoid and dermatitis herpetiformis confirmed earlier reports. Chronic bullous dermatoses of childhood was seen in 4 patients, all of whom had total remission within one year of onset disease.
The characteristics of three primary bullous diseases, pemphigus, pemphigoid, and dermatitis herpetiformis, seen in this country, probably reflecting this region, are discussed and compared to those reported in the literature in the West. One hundred and forty-eight patients with bullous diseases were seen over a period of 15 years. The criteria for confirmation of clinical diagnosis were the findings of the direct immunofluorescent test. Pemphigus vulgaris was the commonest bullous disease. The incidence of bullous pemphigoid was highest in the Indians of Malaysian origin, and they are also more likely to develop pemphigus vulgaris than any other ethnic group. Linear IgA type formed the entire group of dermatitis herpetiformis. The granular type was not seen at all. The patients were treated with prednisolone alone or together with methotrexate or azathioprine. Dapsone alone controlled the dermatitis herpetiformis. The known association between pemphigus and other diseases with immunologic disturbances was not found in this study. The natural history of the bullous disease as seen in the follow-up patients is described. Deaths in pemphigus and pemphigoid were more due to either complications of steroid therapy or unassociated diseases than the primary disease itself. Introduction of immunofluorescence as a diagnostic tool in pemphigus and extension of this facility to other bullous diseases has led to detailed characterization of these diseases as seen in the West; however, publications in English dealing with epidemiologic aspects of bullous diseases in this region are rare.(ABSTRACT TRUNCATED AT 250 WORDS)