Pemphigus erythematosus. Presentation of four cases and review of literature.

Four patients with pemphigus erythematosus, three male and one female, are presented and the literature is reviewed. One of the patients had penicillamine-induced pemphigus erythematosus. The mean age of onset was 66 years. Direct immunofluorescent examination of perilesional skin demonstrated immunoglobulin and/or complement deposition in both the intercellular cement substance (ICS) of the epidermis and at the dermoepidermal junction (DEJ) in all four patients. The lupus band test was positive in three of the four patients when biopsies from uninvolved skin were studied. Indirect immunofluorescent examination of the sera for anti-ICS antibodies was positive in all patients. All four patients had positive antinuclear antibody tests (ANA), but anti-deoxyribonucleic acid (DNA) and anti-extractable nuclear antigens (ENA) were negative. One patient had a positive rheumatoid factor. Serum creatinine and total complement were normal in all four patients. The patients were treated with various combinations of oral corticosteroids, topical corticosteroids, dapsone, and oral therapy, which consisted of hydrogen peroxide, benadryl elixir, and decadron elixir. Three patients were in complete remissions and the fourth patient was in incomplete remission after 4 to 10 months of follow-up (mean, 6 months). Patients with pemphigus erythematosus require significantly lower doses of systemic corticosteroids for control of disease. Dapsone is an important agent for adjuvant therapy and facilitates use of lower doses of systemic corticosteroids.