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A case of severe leucocyte adhesion deficiency occurred in a 6 1/2-month-old boy whose parents were first-degree cousins. Evidence of the disease first became apparent with the late separation of the umbilical cord on the 20th day and with the later development of omphalitis. The most specific finding was the very low levels of CD18 and CD11, 0.44 and 0.15%, respectively. The boy died from sepsis which occurred as an extension of necrotic lesions on the ear and in the gluteal area.
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