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A child with Kohlerâs disease may present similarly to a child with a foot injury. An examination will reveal pain and tenderness over the dorsomedial midfoot, with or without local skin changes. Affected children typically walk with a limp or antalgic gait, with weight bearing on the lateral side of the foot.
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.