PG is a rare disease, with the ulcerative variant being most frequent. The lower legs are the most commonly affected sites. The recurrence rate in our study was about 46% regardless of the treatment prescribed. Pulmonary involvement was fatal in two patients.

No abstract

Our patient showed some distinctive findings never reported previously in RIP: a histological focal keratinocyte necrosis, and the presence of autoantibodies reacting with a 110 kDa keratinocytic protein in immunoblot analysis. Because of a different prognosis, it is important to differentiate RIP and paraneoplastic pemphigus (PNP), although cases of ionizing radiation-induced PNP had also been described. As in our patient, RIP seems to respond well to systemic corticosteroids and immunosuppressive therapy, which induce remission within a few months.

Neonatal pemphigus vulgaris is a rare autoimmune disease that is caused by transplacental passage of pemphigus vulgaris autoantibodies. The association of maternal pemphigus vulgaris with neonatal disease pemphigus vulgaris has been only rarely reported. We describe an infant with pemphigus vulgaris born to a mother whose disease was in remission.