CGD is a rare phagocyte defect with an incidence of four to five per million individuals, caused by genes affecting one X-linked and three autosomal recessive chromosomes. It is associated with recurrent abscesses of different size and locations. We present an 8 year old girl who developed large abscesses at her thigh since 2 years of age.
Key Words : chronic granulomatous disease (CGD), recurrent abscess, immunodeficiency disorders, gamma interferon
The patient is an 8 year old girl who developed a large abscess at her thigh just at the site of childhood vaccinations when she was 2 years of age. The large 4×5-sized abscess contained voluminous yellow-greenish pus which led to persistent drainage. Many topical and systemic antibiotics were used without any result and multiple courses of hospitalizations with intravenous use of wide-spread antibiotics had no benefits. Gram stain and smear always showed a mixture of microbial agents for instance staphylococcus, either S. alba or S.aureus. The abscess had 2 to 4 stoma apart from each other and sometimes locolation of pus posed the need to incision and drainage of mutiple sites. Once acid fast bacilli were grown and anti tubercolosis medications were prescribed and used for at least 9 months without any significant relief. Because of refractoriness of abscesses , phagocyte defect were sugessted and an immunological consultation performed when she was 6 years of age. Immunoglobulines were within normal limits. Nitro Blue Tetrazolium (NBT) and Di Hydro Rhodamine test (DHR) was performed and showed a significant defect of respiratory burst and the patient regarded as " Chronic Granulomatous Disease". Long term therapeutic doses of Trimetoprim-Sulfisoxazole followed by continous prophylaxis, resulted to some relief. Introduction of Gamma- interferon as an every-other-day schedule were terminally led to closure of abscess stoma and terminated the discharge. Multiple scars of previously draining abscesses are visible at her thigh. There was no other site of local or systemic infection and the general status remained good during and after Gamma- interferon therapy.