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The authors report a case of Woringer-Kolopp syndrome. This was a rare form with multiple cutaneous features with a typical clinical and histological appearance. Ultrastructural study revealed the presence of granules with an opaque centre in the cytoplasm of the abnormal cells, and the close relationship of the latter with neurites. On the basis of these characteristics, they may be likened to the Merkel cells of the epidermis. Thus Woringer-Kolopp disease could be related to the proliferation of Merkel cells.
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