Lupus Pernio

Lupus Pernio presented by Jennifer Armstrong
Grand Rounds, USC Dermatology
May 3, 2011

1. Grand Rounds USC Dermatology May 3, 2011 JENNIFER ARMSTRONG Lupus Pernio

2. Overview Sarcoidosis is a multisystem granulomatous inflammatory disease that can affect any organ. Cutaneous lesions present in 20%-35% of patients

3. Lupus Pernio Lupus pernio , first described by Besnier in 1889, is a manifestation of sarcoidal skin lesions.

4. Lupus Pernio Its name comes from Latin perniō = chilblain on the foot. Inflammation of the skin of the hands or feet, resulting from exposure to cold Thought to resemble a mild frostbite Lupus Pernio Frostbite

5. Epidemiology More common in African American’s and Puerto Rican populations Female> Male Usually with long-standing systemic, usually pulmonary disease

6. Etiology The etiology of this disease is still unknown. The serum concentration of angiotensin -converting enzyme (ACE) is increased, and measurements have been used as an index of disease activity.

7. Cutaneous Findings: violaceous , indurated plaques and nodules ………………………………………………………Ears …..…………………………………………………………………….Nose ………………………… Dorsum of hand, fingers ……………………………….………… Toes …………………………………Cheeks and Lips

8. Organs Involved ………………..…………Chronic Uveitis/Occular lesions 37% …….……..….. Intrathoracic Involvement in 74% ………………………………..Bone Cysts 43%

9. Key Findings Cutaneous involvement is either specific or nonspecific. Specific lesions manifest as noncaseating granulomas that consist of mononuclear phagocytes, epithelioid macrophages and multinucleate giant cells Whereas nonspecific lesions (EN) do not reveal granulomas on histopathologic examination.

10. Standard Treatment A stepwise approach to patient care is appropriate First Line: mild skin-limited disease. P otent topical corticosteroids clobetasol I ntralesional injections triamcinolone (3-10 mg/ mL ) First Line: deforming skin lesions or for widespread disease S ystemic therapy: prednisone 40-80 mg/ day tapered used alone or in combination with antimalarials or methotrexate

11. Standard Treatment Second Line Antimalarials and methotrexate may be used as monotherapy for steroid-resistant sarcoidosis or in patients unable to tolerate steroids. Given the concern regarding ocular toxicity, the maximum dosages of chloroquine and hydroxychloroquine should not exceed 3.5 and 6.5 mg/kg/day, respectively. Methotrexate is given in weekly doses of 10-30 mg

12. Treatment – Chronic/Refractory TNF - α antagonists I nfliximab ( IV 3 -10 mg/kg at 0, 2 and 6 weeks) Etanercept (injected subcutaneously at doses of 40 mg either weekly or every 2 weeks) Thalidomide may have a role in cutaneous sarcoidosis , especially in refractory and chronic cases that are resistant to the standard regimens . 50 to >400 mg/ day (average 100mg/ day) has limited, but promising supporting data

13. Other Isotretinoin , 0.5-2 mg/kg/day, has been used successfully in a handful of reported cases . Ablative: Pulsed dye or CO2 laser is available for the debulking of granulomatous lesions However, there are no evidence-based recommendations because of the limited number of patients treated Melatonin (20 mg/day) and allopurinol (100-300 mg/day) are not well studied in cutaneous sarcoidosis , and the clinical experience with tetracycline derivatives has been mixed.

14. Prognosis The course is usually chronic, and severe cosmetic disfigurement may result. Lupus pernio , especially involving the nasal rim, has pulmonary involvement upper respiratory tract (50%) lungs (75%).

15. Differential Diagnosis Lupus vulgaris Deep Fungal Lupus erythematosus Rhinophyma when localized to the nose Malignant pleomorphic lymphoma Protracted superficial Wegener's granulomatosis Tertiary syphilis Discoid Lupus

16. References Chensue SW, Warmington K, Ruth J, Lincoln P, Kuo MC, Kunkel SL: Cytokine responses during mycobacterial and schistosomal antigen-induced pulmonary granuloma formation. Production of Th1 and Th2 cytokines and relative contribution of tumor necrosis factor. Am J Path 1994, 145:1105-1113 Badgwell C, Rosen T: Cutaneous sarcoidosis therapy updated. J Am Acad Dermatol 2007, 56:69-83 Shakoory B, Chathman W. Recognizing and managing the musculoskeletal manifestations of Sarcoidosis . The Journal of Musculoskeletal Medicine 2008, 25: 12   Baughman RP, Lower EE . Newer therapies for cutaneous sarcoidosis : the role of thalidomide and other agents . Am J Clin Dermatol . 2004;5(6):385-94 . Yayoi NAGAI, Naoya IGARASHI, Osamu ISHIKAWA. Lupus pernio with multiple bone cysts in the fingers. The Journal of Dermatology Volume 37, Issue 9, pages 812–814, September 201

17. References cont. James DG. Lupus pernio . Lupus . 1992 May;1(3):129-31 Fernandez-Faith E, McDonnell Cutaneous sarcoidosis : differential diagnosis. J.Clin Dermatol . 2007 May-Jun;25(3):276-87. Takashi Koyama, MD, Hiroyuki Ueda, MD, Kaori Togashi , MD, Shigeaki Umeoka , MD, Masako Kataoka , MD and Sonoko Nagai, MD. Radiologic Manifestations of Sarcoidosis in Various Organs. RadioGraphics . March-April 2011, 31 (2 ) Marchell , Richard M; Judson, Marc. A Cutaneous Sarcoidosis Semin Respir Crit Care Med 2010; 31: 442- 451 Doherty CB, Rosen T . Evidence-based therapy for cutaneous sarcoidosis . Drugs . 2008;68(10):1361-83 . PICS: TheAltitudeExperience.com , Dermis.net , dermatlas , Journal of Head and Neck Medicine, Lexic , emedicine , Access Medicine