Upper dermal elastolysis: a comparative study with mid-dermal elastolysis

We encountered a patient who complained of many small papules on the neck, shoulders, upper chest and upper back. Biopsy specimens showed complete loss of elastic fibers in the upper dermis including papillary dermis, whereas those of the mid dermis were intact. Electron microscopy revealed that assembly of component fibrils of elastic fibers was loose, and electron dense substance was aggregated in the spaces between these loosely bound subunit fibrils or along the periphery of abnormal fibers. Dermal phagocytes engulfed abnormal as well as normal elastic fibers. Upper dermal elastolysis is a clinical and histopathological entity different from mid-dermal elastolysis. Ultrastructural changes of the former are essentially similar to those of the latter but much more severe. It is suggested that activated elastophagocytosis of dermal phagocytes may play a role in this disease.