Neonatal Pemphigus Vulgaris: A Case Report

Pemphigus vulgaris (PV) is an autoimmune blistering disorder of the skin and mucous membranes. The transplacental passage of maternal immunoglobulin G (IgG) autoantibodies to desmoglein-3 (a transmembrane glycoprotein component in the skin) from the mother's blood to the fetus can cause transient PV in the neonatal period. The duration of PV is short in the neonatal period, and the disease is improved with no prolonged sequelae. The similarity of skin lesions in PV to other skin conditions, such as infectious diseases caused by bacterial, viral, and fungal pathogens, or inherited bullous disorders, such as epidermolysis bullosa and incontinentia pigmenti, leads to misdiagnosis, inappropriate hospital admission, and poor antimicrobial treatment of patients. On the other hand, the maternal history of PV, besides laboratory examination, confirms the exact diagnosis. In this case report, we present a male term neonate with multiple pustules and blisters on the skin, developed within the first hours of life. The patient was admitted to the neonatal ward of our hospital for a sepsis workup and antibiotic treatment. Regarding the positive maternal history of PV in the second trimester of pregnancy and neonatal examinations skin biopsy confirmed the diagnosis of this disease.