– Granuloma formation (superificial to mid dermis) distributed peripherally to a central zone of dermal atrophy and loss of elastic tissue. Elastophagocytosis. Unusual late presentation of X-linked chronic granulomatous disease in an adult female with a Adult onset X-linked chronic granulomatous disease. Chronic granulomatous disease (CGD) is a rare primary immunodeficiency characterized by recurrent life-threatening bacterial and fungal infections, granuloma formation and intestinal disease. CGD is a recessive genetic disorder caused by a defect in one of five genetic loci coding for NADPH oxidase, a critical enzyme required in the phagocytosis .