Unusual direct immunofluorescence (DIF) pattern in pemphigus herpetiformis- A case report

Pemphigus herpetiformis (PH) is an autoimmune intraepithelial bullous skin disorder. A 61-year-old female presented with history of multiple pruritic erosions, ulcers all over body, and diffuse loss of hair over scalp. Oral and genital mucosas were uninvolved. Subcorneal separation with suprapapillary thinning of epidermis, neutrophilic spongiosis, and elongation of rete ridges were seen on histopathology. Direct immunofluorescence (DIF) revealed IgG deposits in intercellular zone in fish net like pattern and focal linear IgA deposits along basement zone. Indirect immunofluorescence (IIF) revealed antibodies to desmoglein1 (Dsg-1) positive. A final diagnosis of PH was given. The patient responded well to treatment with dapsone and steroids.