A 34-year-old female patient presented with the classical features of keratosis lichenoides chronica, a very rare dermatosis. Typical hyperkeratotic papules in a linear and reticular pattern were located on the upper extremities and the trunk. The nails and the perionychial areas showed marked vegetating lesions. After administration of a combined retinoid-PUVA therapy impressive remission was observed. The clinical picture, the differential diagnosis, in particular from lichen ruber planus, and the therapeutic aspects are discussed.
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