Frequency
Age
Sex
Race
Pemphigus herpetiformis is a rare clinical variant of pemphigus. Frequency of occurrence remains undetermined. Although frequency of occurrence of pemphigus herpetiformis is not determined, pemphigus herpetiformis has been reported in Europe, Japan, and the United States. In a large study conducted in Eastern Europe, 15 patients (7.3%) with pemphigus herpetiformis were found among 205 patients with pemphigus. In a smaller study conducted in Italy, 5 patients with pemphigus herpetiformis were found among 84 patients with pemphigus. Therefore, pemphigus herpetiformis accounts for approximately 6-7% of pemphigus in European populations.
Because pemphigus herpetiformis is rare, ethnic distribution is not determined yet. Because pemphigus herpetiformis occurs in the United States, Europe, and Asia, it does not appear to have a specific ethnic predominance.
Because pemphigus herpetiformis is rare, sex distribution has yet to be defined clearly. Studies in the literature do not appear to support a sex predilection.
The age of onset for pemphigus herpetiformis ranges from 30-80 years, with a mean age of onset of 60 years. Moutran et al describes an unusual case of onset at age 6 years successfully treated with dapsone. One case report describes transplacental transmission of pemphigus herpetiformis to an otherwise healthy infant with subsequent resolution after age 3 weeks.
