OC11Hyperimmunoglobulin E syndrome presenting with oral premalignant papillomas

Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency characterized by the clinical triad of recurrent staphylococcal abscesses, recurrent cyst-forming pneumonia and an elevated serum IgE level. A 10-year-old female with HIES presenting with widespread oral premalignant papillomas, which are an uncommon co-existing manifestation, and labial herpes simplex virus infection is reported. The patient experienced recurrent furuncles, pneumonia, tuberculosis, otitis media and sinusitis attacks since she was 6 months-old. She had been diagnosed with HIES at the age of 6 when the papillomatous lesions first occurred in oral cavity. Family history was not remarkable. Intraoral examination revealed diffuse multiple papillomas covering the tongue, lips, buccal, labial and palatinal mucosa and gingiva extending to the crowns of teeth. The lips were enlarged to the double of normal size due to numerous papilloma formations and the outline could hardly be traced. These lesions caused nutritional, aesthetic, psycological problems besides loss of appetite. Histopathological examination of the lesions revealed papillary proliferations of squamous epithelium which was confirmed to be positive for human papilloma virus DNA by molecular based test (Inno lipa HPV genotyping test) which is based on reverse hybridization principle and PCR. Typing of HPV was found to be as HPV 6, 11, 16, 18, 31, 33, 35, 39, 40, 42–45, 51–53, 56, 58, 59, 66, 68, 70 and 74, forming a high/intermediate risk group for malignancy. For the management of widespread papillomas, diode laser was applied. After therapy, significant clinical improvement, reduction in oral discomfort and improvement in appetite was observed. Since infection with HPV is a significant independent factor for oral squamous cell carcinoma, the patient remains under regular review.