Background/Aims: Hypertrophic lupus erythematosus (HLE) produces not only disfiguring and long-lasting skin changes, but also proves to be particularly resistant to therapy. In a patient with a disease refractory to conventional therapy, we observed a predominantly CD4+ T cell infiltrate that we sought to target therapeutically. Methods: The patient was treated with the CD11a-directed monoclonal antibody efalizumab together with 10 mg isotretinoin and 5 mg prednisone. Results: Within 6 months, the inflammatory hyperkeratotic plaques were almost completely healed, with minimal residual erythema and scarring. The cutaneous lupus activity and severity index score decreased from 8 to 4 of a total of 14 points, and pseudoepitheliomatous hyperplasia and CD4 T cell infiltrates within the lesions were reduced. Conclusion: HLE features interface dermatitis and epidermal hyperplasia, which are both explainable by T-cell-mediated immunologic effects. Correspondingly, our case responded well to the treatment with efalizumab. While the withdrawal of efalizumab from the market leaves patients with psoriasis many other options for effective therapy, it disproportionately affects patients with T-cell-mediated orphan diseases like refractory HLE.

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