Pemphigus Syphiliticus: A Case Report from Italy and a Comprehensive Literature Review

Background: Congenital syphilis remains a significant global health concern, with severe morbidity and mortality if undiagnosed and untreated. Although many infants appear asymptomatic at birth, subtle clinical signs—including bullous lesions (pemphigus syphiliticus)—may facilitate early detection. Recognizing this rare manifestation is crucial for timely intervention, reducing serious outcomes. Methods: We systematically reviewed Medline (PubMed), Embase, and the Cochrane Central Register of Controlled Trials from inception to November 2024 for cases of con-genital pemphigus syphiliticus. We extracted demographic, clinical, laboratory, radio-logical, treatment, and outcome data. Additionally, we included clinical information from a newly documented case of congenital pemphigus syphiliticus managed in our center. Results: Nineteen cases of congenital syphilis with bullous lesions were identified, 18 with sufficient detail for analysis. Patients presented three distinct clinical patterns: confined palmoplantar lesions, acrally distributed lesions, and diffuse bullous-erosive involve-ment. Despite variable severity, cutaneous manifestations provided a key diagnostic clue. Nontreponemal and treponemal serologic tests were central to diagnosis, supported by maternal screening and imaging. Intravenous penicillin G was the most frequently employed therapy. While most infants achieved remission, severe respiratory in-volvement was associated with mortality. Our new case aligned with these findings, demonstrating full resolution after appropriate antibiotic therapy. Conclusions: Pemphigus syphiliticus, though rare, is an important early sign of congenital syphilis. Prompt recognition and diagnosis—enabled by diligent maternal screening, targeted neonatal testing, and careful clinical examination—are essential to initiate timely penicillin therapy and prevent severe complications or death. This review underscores the need for heightened clinical vigilance and adherence to established guidelines for syphilis screening and treatment during pregnancy, ultimately improving neonatal outcomes.