Angina bullosa hemorrhagica an enigmatic oral disease

Abstract

Angina bullosa hemorrhagica (ABH) is an enigmatic oral disorder described for the first time by Badham in 1967 to define blisters with a hematic content in the oral cavity and oropharynx unrelated to any hematological, dermatological or systemic disease. The ABH is an uncommon disease of the oral cavity distinctively affecting adults, with the highest incidence over the 5th decade of life. This process is considered nowadays to have a multifactorial etiopathogenesis, where mild oral traumatisms can trigger the blisters in susceptible individuals. Certain association on the onset of the lesion with the chronic use of inhaled steroids and, more controversially, with triggering systemic disorders, such as, diabetes or hypertension has been described. Characteristically, the ABH blisters are acute and are located on the lining mucosa, more frequently on the soft palate. Usually, the lesions are solitary and rupture easily, resulting in a superficial ulceration that heals quickly without scarring. The histopathological analysis shows a subepithelial blister containing blood and direct immunofluorescence on the epithelium is negative. The differential diagnosis should consider all oral vesiculobullous disorders with hematic content, including mucocutaneos, hematological or cystic pathology. The diagnosis of ABH is clearly clinical, although the biopsy might be helpful on atypical or abnormally recurrent cases. The general prognosis of ABH is good and the treatment is symptomatic. Although it is an uncommon disease, the angina bullosa hemorrhagica should be considered in the differential diagnosis of oral vesiculo-bullous processes. Acknowledging this entity will help in differentiating it from important mucocutaneous and hematological diseases such as pemphigus vulgaris, mucous membrane pemphigoid or coagulation disorders. In this review we analyze the main etiopathogenic, clinicopathological, diagnostic and therapeutic aspects of this enigmatic oral condition.