Pemphigus foliaceus: a case with serologic features of Senear-Usher syndrome and other autoimmune abnormalities

The two variants of superficial pemphigus, pemphigus erythematosus (Senear-Usher syndrome) and pemphigus foliaceus, share common histopathologic and indirect immunofluorescence findings. They differ in that pemphigus erythematosus is limited in distribution and usually has concomitant basement membrane zone deposition of immunoglobulin and complement in lesional skin in addition to intercellular space staining in the epidermis, whereas pemphigus foliaceus is a generalized process that reputedly lacks basement membrane zone staining. A 49-year-old man with an array of immunologic abnormalities, including pernicious anemia and Sjögren's syndrome consistent with broad immunologic dysfunction, developed generalized superficial pemphigus (pemphigus foliaceus) with concomitant basement membrane zone immunoreactant deposition. The demonstration of basement zone immunofluorescent staining lends additional support to the basic viewpoint that pemphigus erythematosus and pemphigus foliaceus are variable phenotypic expressions of the same basic disease process.