IgG/IgA pemphigus with dyskeratotic acantholysis and intraepidermal neutrophilic microabscesses

Herpetiform vesicles and erythema with erosion developed on the trunk of a 48‐year‐old Japanese man. Acantholysis was observed in the spinous layer of the lesional epidermis and direct immunofluorescence revealed cell surface deposition of immunoglobulin IgG and C3. Indirect immunofluorescence could not detect circulating anti‐cell surface antibodies. Immunoblot analysis detected neither anti‐desmoglein (Dsg)1 antibody nor anti‐Dsg3 antibody. Enzyme‐linked immunosorbent assay could detect IgG and IgA anti‐Dsg1 antibodies and IgA anti‐Dsg3 antibody, in addition to a gray zone titer of IgG anti‐Dsg3 antibody. Intraepidermal neutrophilic infiltration with neutrophilic microabscesses and intense dyskeratotic cells were histopathologically characteristic in this case. Skin lesions improved within 1 month and remission has continued for 9 years under oral administration of dapsone.

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