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[Chronic granulomatosis: a rare differential diagnosis in liver granulomas in adulthood]
J Glaser1, M Gahr, A munnethal
1Medizinische Klinik I, Städtische Kliniken Kassel.
Abstract:
A now 43-year-old man was known since childhood to have mesenteric and subcutaneous lymphadenopathy. Histological examination of liver biopsies and excision of some lymph nodes when an adult showed epithelioid granulomas, in places with Langhans giant cells. Diagnostic splenectomy revealed no pathological findings. His present admission to hospital was for an infection with high fever. On auscultation moist rales were audible over the apex of the left lung. The chest radiography showed pneumonic infiltration. Blood culture grew Pseudomonas aeruginosa. Ultrasound demonstrated hypoechogenic homogeneous and smoothly circumscribed round foci in the liver hilus and around the coeliac trunk. The upper lobe pneumonia healed under antibiotic treatment. As chronic granulomatosis was suspected, the nitroblue tetrazolium and superoxide production tests were performed. They demonstrated that the capacity of the granulocytes to form oxygen radicals was markedly diminished. Chronic granulomatosis is an inherited disorder of granulocyte function linked to the X-chromosome. It must be included in the differential diagnosis of any unclear granulomatous disease even in adults.