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Kohlerâs disease is a rare condition in which the tarsal navicular bone (a bone in the arch of the foot) becomes inflamed. This bone disease is named after the German radiologist who described the condition in the early twentieth century. Kohlerâs disease usually affects children between the ages of three and five. It occurs more commonly in boys than in girls, and is often unilateral, affecting one foot. It is described as an avascular necrosis, or osteonecrosis, in which bone tissue deteriorates due to an interruption of blood supply.
Raw skin may provide a portal of entry for germs, leading to sepsis (bloodstream infection). There are often draining ears, swollen glands, enlargement of the liver and spleen, liver disease, anemia and neutropenia (low white blood cells). Loss of appetite, irritability, failure to thrive, and respiratory symptoms may occur. Because of their appearance, these children may be misdiagnosed as abused. Letterer-Siwe disease is the most severe form of Langerhans cell histiocytosis.