Chest
Volume 135, Issue 2, February 2009, Pages 468-476
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Original Research
Sarcoidosis
The Treatment of Lupus Pernio: Results of 116 Treatment Courses in 54 Patients

https://doi.org/10.1378/chest.08-1347Get rights and content

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.

Section snippets

Materials and Methods

This study was approved by the Medical University of South Carolina (MUSC) Institutional Review Board.

Results

Ninety-two patients with lupus pernio were identified from the sarcoidosis database. Nine patients had concomitant severe extracutaneous sarcoidosis (cardiac sarcoidosis, neurosarcoidosis, and severe pulmonary sarcoidosis) that was the primary reason for therapy and so were excluded from this analysis. Eleven patients had only a single visit and, therefore, the effect of therapy could not be assessed. Two patients had a history of lupus pernio but no active lesions and were not treated. No data

Discussion

Our study is clearly the largest to examine the treatment of lupus pernio with 116 separate treatment courses analyzed in 54 individual patients. The largest previous series13 of treatment of lupus pernio that we identified reported 30 patients. However, those authors13 mentioned that only 1 of their 30 patients had lupus pernio, although we believe that most of them had lupus pernio by virtue of their description of the skin lesions. In addition, the authors13 did not provide their methodology

Acknowledgment

The authors thank Dr. Sooyeon Kwon for assistance with data analysis.

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      Citation Excerpt :

      This is particularly true for pulmonary sarcoidosis, where in one series, all 36 patients with an acute exacerbation of pulmonary sarcoidosis had a return of spirometry and symptoms to baseline after a median of 3 weeks of prednisone at a dose of 20 mg/day [3]. True corticosteroid failure is more common with the lupus pernio form of skin sarcoidosis [4], neurosarcoidosis [5,6], and cardiac sarcoidosis [7–10]. As is implied above, true corticosteroid failure appears to be somewhat organ dependent.

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    1

    Drs. Stagaki, Mountford, and Lackland have no conflicts of interest to disclose.

    2

    Dr. Judson has received research grants from Celgene and Centocor.

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