The treatment of lupus pernio: results of 116 treatment courses in 54 patients

Chest. 2009 Feb;135(2):468-476. doi: 10.1378/chest.08-1347. Epub 2008 Sep 23.

Abstract

Background: Lupus pernio is a disfiguring sarcoidosis skin lesion that is difficult to treat and often causes a major psychosocial impact that may adversely affect the patient's quality of life. We reviewed the treatment outcome of 54 patients with lupus pernio who received 116 individual courses of treatment in our sarcoidosis clinic.

Methods: Lupus pernio patients were identified from an institution-approved database. All patients were assessed at each clinic visit with facial photographs. By examining the photographs, the percentage of face involved (< 10%, 10 to 25%, > 25 to 50%, > 50%) was determined as was the effect of therapy (resolution, near resolution, improvement, no change, worsening). Medications included infliximab-containing regimens; systemic corticosteroids; noninfliximab, noncorticosteroid agents; and corticosteroids plus noncorticosteroid agents.

Results: In terms of achieving resolution or near resolution, infliximab regimens were superior to all others (infliximab, 77%; corticosteroids plus noncorticosteroids, 29%; corticosteroids, 20%; noncorticosteroids, 11%; infliximab vs other therapies: corticosteroids plus noncorticosteroids, p = 0.0015; corticosteroids, p = 0.0005; noncorticosteroids, p = 0.0002). The percentage of facial involvement also improved most with infliximab. Evaluating a secondary analysis of achieving resolution, near resolution, or improvement, infliximab (92%) was superior to noncorticosteroids (20%; p < 0.0001) and corticosteroids plus noncorticosteroids (56%; p = 0.0098), but not corticosteroids (72%; p = 0.2456); and noncorticosteroid agents were inferior to all other regimens.

Conclusions: Infliximab appears superior to systemic corticosteroids with or without additional agents for the treatment of lupus pernio. Noninfliximab, noncorticosteroid-containing regimens are of limited use for this condition.

Publication types

  • Comparative Study

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Adult
  • Antibodies, Monoclonal / therapeutic use*
  • Cohort Studies
  • Disability Evaluation
  • Dose-Response Relationship, Drug
  • Drug Administration Schedule
  • Drug Therapy, Combination
  • Female
  • Follow-Up Studies
  • Humans
  • Infliximab
  • Male
  • Middle Aged
  • Probability
  • Registries
  • Retrospective Studies
  • Risk Assessment
  • Sarcoidosis / diagnosis
  • Sarcoidosis / drug therapy*
  • Severity of Illness Index
  • Skin Diseases / diagnosis
  • Skin Diseases / drug therapy*
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Antibodies, Monoclonal
  • Infliximab