INTRODUCTION:
lgA pemphigus is a rare, chronic,
relapsing, benign group of autoimmune
intraepidermal blistering dermatosis with an
unknown etiology. It is characterized by
significantly pruritic, vesiculopustular lesions that
occur mainly on the trunk and proximal extremities.
Histopathologic and immunofluorescence studies
show intraepidermal blisters and deposition of
immunoglobulin A in the intercellular spaces of the
epidermis, respectively.
CASE REPORT
To our knowledge, we present the first
reported pediatric case of lgA pemphigus,
intraepidermal neutrophilic type, in an 8-year old
Filipino female with a 2-year history of generalized
papules and flaccid pustules, some forming an
annular pattern. Diagnosis was confirmed by
histopathology and direct immunofluorescence.
Enzyme-linked immunosorbent assay for
Desmoglein 1 was negative. Complete clearance of
lesions was achieved with dapsone, colchicine and
prednisone.