Familial benign pemphigus, or Hailey-Hailey disease (HHD), is a rare (1 in 50,000), benign, autosomal dominant cutaneous disorder that causes a painful rash and blistering commonly occurring in the intertriginous folds. Despite having a good prognosis, there is no cure for HHD and the disease can be quite debilitating to the quality of life.
The complexity of HHD can be compounded by superimposed eczema herpeticum (EH) or Kaposi's varicelliform eruption, which is caused by a viral infection occurring in preexistent cutaneous conditions. We present a unique clinical presentation of HHD with superimposed EH caused by herpes simplex virus type 2 (HSV-2) infection managed in a burn unit. It is highly advised that a recalcitrant HHD with superimposed EH caused by HSV-2 infection should be managed in burn centers that offer multimodalities for prompt, rigorous management. Early diagnosis and treatment are highly suggested for EH to avoid fatal complications.
Copyright © 2019, Chin et al.
1 General Surgery, St. John's Episcopal Hospital, Far Rockaway, USA.
2 Surgery, Burn Center, The Johns Hopkins University School of Medicine, Baltimore, USA.
3 Plastic Surgery, The Johns Hopkins University School of Medicine, Baltimore, USA.