Nail changes occur in about 25% of systemic lupus erythematosus (SLE) cases. Onycholysis has been reported as the most frequent abnormality in SLE. Nailbed hyperkeratosis may be observed in both SLE and discoid lupus erythematosus (DLE). Involvement of the nail apparatus in DLE is extremely uncommon and never restricted to it. We report on a patient in whom the clinical features on the proximal nailfold were similar to those observed on the skin of a patient with typical DLE. This has, to the best of our knowledge, not yet been reported. The patient also exhibited a very distinctive prominent subungual hyperkeratosis. Interestingly, the patient developed biological alterations suggesting a systematization of the disease. Only a combination of systemic corticoids, retinoids and antimalarials was able to achieve nail improvement and this partial resistance to therapy may be explained by the very unusual subungual hyperkeratosis.