title: Control of widespread hypertrophic lupus erythematosus with T-cell-directed biologic efalizumab.
abstract: 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.
authors.affiliation: Department of Dermatology, University Hospital of Zürich, Zürich, Switzerland.
authors.forename: Alexander A
authors.initials: AA
authors.lastname: Navarini
authors.forename: Katrin
authors.initials: K
authors.lastname: Kerl
authors.forename: Lars E
authors.initials: LE
authors.lastname: French
authors.forename: Ralph M
authors.initials: RM
authors.lastname: Trüeb
mesh_terms: 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
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D003879:Dermatologic Agents; D015474:Isotretinoin; D011241:Prednisone; C472181:efalizumab