Congenital penoscrotal lymphedema complicated by sepsis associated with a streptococcal infection

Congenital lymphedema is a relatively rare disease caused by congenital abnormality of the lymphatic system. Although bacterial infection frequently causes complications with lymphedema, severe sepsis in congenital lymphedema of the genitalia has not yet been reported. We describe a patient with congenital penoscrotal lymphedema complicated by cellulitis, lymphangitis, and severe sepsis associated with a streptococcal infection. This case represents the importance of obtaining a detailed clinical history and physical findings.