A new case of typical mid dermal elastolysis was studied with electron microscopy. Elastic fibers were irregularly branched or lumpy, but subcomponents of these fibers were often normal. In some fibers, loose assembly of skeleton fibrils and aggregation of dense substances were observed. Phagocytosis of normal and abnormal elastic fibers by dermal macrophages was observed.
This is a case report of multiple lentigines (Leopard Syndrome) with Chiari malformation, i.e. a herniation of the cerebellum into the foramen magnum. This male patient had generalized multiple lentigines since birth, and the lesions spread steadily, involving the scalp but sparing all mucous membranes. Organ system involvements included heart murmur, ocular hypertelorism, and retardation of growth.
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