Lupus Pernio

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Kenneth S. Alpern - One of the best experts on this subject based on the ideXlab platform.

David J. Schreiner - One of the best experts on this subject based on the ideXlab platform.

Matthew M. Goodman - One of the best experts on this subject based on the ideXlab platform.

Joseph L. Jorizzo - One of the best experts on this subject based on the ideXlab platform.

Robert P. Baughman - One of the best experts on this subject based on the ideXlab platform.

  • Sarcoidosis patient with Lupus Pernio and infliximab-induced myositis: Response to Acthar gel.
    Respiratory medicine case reports, 2015
    Co-Authors: Ying Zhou, Elyse E. Lower, Robert P. Baughman
    Abstract:

    Infliximab is an effective treatment for sarcoidosis patients with persistent disease despite glucocorticoids and immunosuppressive therapy. Patients receiving infliximab can experience side effects, inducing an autoimmune reaction. Treatment is unclear for sarcoidosis patients who develop autoimmune reactions to infliximab. We report a case of a patient with advanced sarcoidosis who developed a myositis type reaction to infliximab characterized by diffuse muscle achiness and weakness and marked elevations in serum creatinine phosphokinase (CPK) and aldolase. Manifestations of sarcoidosis and myositis improved after Acthar treatment. This is the first report of successful treatment with Acthar in a patient with advanced sarcoidosis with an autoimmune reaction to infliximab.

  • Features of sarcoidosis associated with chronic disease.
    Sarcoidosis vasculitis and diffuse lung diseases : official journal of WASOG, 2015
    Co-Authors: Robert P. Baughman, Elyse E. Lower
    Abstract:

    Background: The clinical outcome of sarcoidosis is variable. Several features have been proposed as predictive of chronic sarcoidosis. Objectives: To examine the predictive role of clinical features on the clinical outcome score (COS) of sarcoidosis patients five years after initial diagnosis. Methods: A cohort of newly diagnosed sarcoidosis patients seen at one clinic were re-evaluated five years after initial diagnosis. The COS was determined at this five year time point and compared to initial age, race, sex, forced vital capacity (FVC) and chest roentgenogram. We also compared COS to whether patients had Lofgrens syndrome, Lupus Pernio, or cardiac disease and what treatment they received during the five years of observation. Patients with neurologic disease were divided into those with seventh cranial nerve paralysis alone versus those with other neurologic disease (CNS). Results: Of the 335 newly diagnosed patients seen over a three year period, 213 (64%) were evaluated five years after initial diagnosis. Of these patients 168 (79%) were still requiring systemic therapy at five years (COS 7, 8, or 9). Seven features were associated with chronic disease: CNS alone (Odds Ratio (OR)=19.24, p 0.05). Conclusion: In our study, the majority of sarcoidosis patients were receiving systemic therapy five years after initial diagnosis. Patients with reduced lung function, neurologic disease, Lupus Pernio, and cardiac disease were more likely to require prolonged treatment

  • Chronic Facial Sarcoidosis Including Lupus Pernio
    American Journal of Clinical Dermatology, 2008
    Co-Authors: Robert P. Baughman, Marc A. Judson, Alvin Teirstein, Elyse E. Lower, Rozsa Schlenker-herceg, Elliot S. Barnathan
    Abstract:

    Background : Facial lesions including Lupus Pernio are often a form of chronic cutaneous sarcoidosis. Objective : To evaluate the intra- and inter-observer consistency of objective measures of chronic facial lesions. Method : This was a retrospective study of patients with chronic cutaneous facial lesions including Lupus Pernio. The lesions were evaluated using two methods. Results : Of the 25 patients studied, 23 were women and 24 were African American. Lungs (24 patients), sinuses (11 patients), and eyes (7 patients) were also affected. The Sarcoidosis Activity and Severity Index (SASI) characterized individual areas of the face, with 95% of the observations being less than 2 points from the median. A facial SASI total gave a score for the entire face and 93.2% of the scores were within 3 points of the median. Conclusion : Patients with sarcoidosis and chronic facial lesions often have lung, sinus, and eye involvement. The SASI is a reproducible scoring system for chronic facial lesions.

  • Chronic facial sarcoidosis including Lupus Pernio: clinical description and proposed scoring systems.
    American journal of clinical dermatology, 2008
    Co-Authors: Robert P. Baughman, Marc A. Judson, Alvin Teirstein, Elyse E. Lower, Rozsa Schlenker-herceg, Elliot S. Barnathan
    Abstract:

    Background: Facial lesions including Lupus Pernio are often a form of chronic cutaneous sarcoidosis.

  • Thalidomide for chronic sarcoidosis
    Chest, 2002
    Co-Authors: Robert P. Baughman, Marc A. Judson, Alvin Teirstein, David R. Moller, Elyse E. Lower
    Abstract:

    Study objectives Thalidomide therapy has been shown to modify granulomatous diseases, such as tuberculosis and leprosy. Lupus Pernio is a skin manifestation of sarcoidosis that does not remit spontaneously, and was used as a marker of efficacy of thalidomide for sarcoidosis. Design An open-label, dose-escalation trial of thalidomide. Setting Patients were seen at one of four specialized sarcoidosis clinics in the United States. Patients Fifteen patients with Lupus Pernio and other manifestations of sarcoidosis unresponsive to prior therapy were enrolled. Interventions Skin lesions were assessed with visual examination by the treating physician, and photographic evaluation by a blinded panel of physicians reviewing photographs of the lesions before and after therapy. Measurements and results Fourteen patients completed 4 months of therapy. All patients experienced some improvement in their skin lesions subjectively, and 10 of 12 evaluable patients showed improvement using photograph scoring. Five patients were better after 1 month (treated with 50 mg/d of thalidomide), seven more patients improved after 2 months (treated with 100 mg/d of thalidomide in the second month), and two patients required an additional month of 200 mg of thalidomide to achieve a response. Patients reported increased somnolence (n = 9), numbness (n = 7), dizziness (n = 2), constipation (n = 6), rash (n = 1), and increasing shortness of breath (n = 1). One patient discontinued therapy because of new-onset dyspnea, due to probably unrelated new-onset congestive heart failure. Conclusion Thalidomide was an effective form of treatment for chronic cutaneous sarcoidosis. The drug was well tolerated and may be a useful alternative to systemic corticosteroids.