Localized pemphigus herpetiformis: two case reports

Pemphigus herpetiformis (PHF) is a rare variant of pemphigus that combines the clinical features of dermatitis herpetiformis and immunopathological features of pemphigus. In previous case reports, distribution of lesions is usually generalized, involving trunk and limbs. We report two cases of localized PHF presenting with grouped pustules on bilateral dorsal feet and left preauricular area, respectively. Microscopic examination showed neutrophilic spongiosis, subcorneal pustules, and focal acantholysis. Both direct immunofluorescence of Case 1 and indirect immunofluorescence of Case 2 revealed moderate deposition of IgG in the intercellular space of upper epidermis. Based on the typical histologic features and immunopathological findings, diagnoses of PHF were made. However, the atypical and localized clinical presentation rendered the process of reaching a diagnosis more difficult. PHF should be added in the spectrum of differential diagnosis of localized pustular disorders.