Control of widespread hypertrophic lupus erythematosus with T-cell-directed biologic efalizumab.
OBJECTIVE
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.


CONCLUSIONS
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.
Navarini
Alexander A
AA
Department of Dermatology, University Hospital of Zürich, Zürich, Switzerland.
Kerl
Katrin
K
French
Lars E
LE
Trüeb
Ralph M
RM
D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized; D015496:CD4-Positive T-Lymphocytes / Q000187:drug effects*; D003879:Dermatologic Agents / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D006801:Humans; D006965:Hyperplasia; D015474:Isotretinoin / Q000627:therapeutic use; D008178:Lupus Erythematosus, Cutaneous / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D003879:Dermatologic Agents; D015474:Isotretinoin; D011241:Prednisone; C472181:efalizumab