Blistering Diseases – Endemic Pemphigus

Endemic Pemphigus, also called Fogo Selvagem, is a very rare blistering disease of the skin that I rarely ever encounter in either my The Woodlands dermatology and Conroe dermatology offices.  Endemic Pemphigus is typically limited to Brazil but with international travel can be seen anywhere in the world.  It is most common in children and young adults.  Clinically, the lesions initially appear as very fragile bulla (blisters) that rupture easily. The lesions eventually become crusted scaly plaques resembling eczema or seborrhea.  Typically, it does not involve the oral mucoas.  It is believed to be triggered as an autoimmune reaction to an antigen from an insect bite with antibodies developing to a key structural protein in the skin called Desmoglein 1.  Treatment is with immunosuppressive medications.