G R A N D R O U N D S
U S C D E R M A T O L O G Y
M A Y 3 , 2 0 1 1
J E N N I F E R A R M S T R O N G
Lupus Pernio
Overview
 Sarcoidosis is a
multisystem
granulomatous
inflammatory
disease that can
affect any organ.
 Cutaneous lesions
...
Lupus Pernio
Lupus pernio, first
described by Besnier in
1889, is a manifestation
of sarcoidal skin lesions.
Lupus Pernio
 Its name comes from Latin perniō = chilblain on the
foot.
 Inflammation of the skin of the hands or feet, ...
Epidemiology
 More common in
African American’s and
Puerto Rican
populations
 Female> Male
 Usually with long-
standing...
Etiology
 The etiology of this disease is still unknown.
 The serum concentration of angiotensin-
converting enzyme (ACE...
Cutaneous Findings: violaceous, indurated plaques and
nodules
………………………………………………………Ears
…..…………………………………………………………………….Nose...
Organs Involved
………………..…………Chronic Uveitis/Occular lesions 37%
…….……..….. Intrathoracic Involvement in 74%
……………………………….....
Key Findings
 Cutaneous involvement is
either specific or nonspecific.
 Specific lesions manifest as
noncaseatinggranulo...
Standard Treatment
 A stepwise approach to patient care is appropriate
 First Line: mild skin-limited disease.
 Potent ...
Standard Treatment
 Second Line
 Antimalarials and methotrexate may be used as monotherapy
for steroid-resistant sarcoid...
Treatment – Chronic/Refractory
 TNF-αantagonists
 Infliximab ( IV 3-10 mg/kg at 0, 2 and 6 weeks)
 Etanercept (injected...
Other
 Isotretinoin, 0.5-2 mg/kg/day, has been used
successfully in a handful of reported cases.
 Ablative: Pulsed dye o...
Prognosis
 The course is usually
chronic, and severe
cosmetic disfigurement may
result.
 Lupus pernio, especially
involv...
Differential Diagnosis
•Lupus vulgaris
•Deep Fungal
•Lupus erythematosus
•Rhinophyma when localized to the nose
•Malignant...
References
• Chensue SW, Warmington K, Ruth J, Lincoln P, Kuo MC, Kunkel SL: Cytokine
responses during mycobacterial and s...
References cont.
 James DG. Lupus pernio. Lupus. 1992 May;1(3):129-31
 Fernandez-Faith E, McDonnell Cutaneoussarcoidosis...
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Lupus Pernio

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

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  • Lupus pernio is usually more common in black women with long-standing systemic, usually pulmonary, sarcoidosis than in other people. It is also commonly seen with chronic uveitis and bone cysts.
  • Red-to-purple or violaceous, indurated plaques and nodules that usually affect the nose, the cheeks, the ears, and the lips, but it can appear on the dorsa of the hands, the fingers, the toes, and the forehead.
  • pulmonary, sarcoidosis than in other people. It is also commonly seen with chronic uveitis and bone cysts. bone cysts in 43% and ocular lesions in 37%.
  • Lupus Pernio

    1. 1. G R A N D R O U N D S U S C D E R M A T O L O G Y M A Y 3 , 2 0 1 1 J E N N I F E R A R M S T R O N G Lupus Pernio
    2. 2. Overview  Sarcoidosis is a multisystem granulomatous inflammatory disease that can affect any organ.  Cutaneous lesions present in 20%- 35% of patients
    3. 3. Lupus Pernio Lupus pernio, first described by Besnier in 1889, is a manifestation of sarcoidal skin lesions.
    4. 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. 5. Epidemiology  More common in African American’s and Puerto Rican populations  Female> Male  Usually with long- standing systemic, usually pulmonary disease
    6. 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. 7. Cutaneous Findings: violaceous, indurated plaques and nodules ………………………………………………………Ears …..…………………………………………………………………….Nose ………………………… Dorsum of hand, fingers ……………………………….………… Toes …………………………………Cheeks and Lips
    8. 8. Organs Involved ………………..…………Chronic Uveitis/Occular lesions 37% …….……..….. Intrathoracic Involvement in 74% ………………………………..Bone Cysts 43%
    9. 9. Key Findings  Cutaneous involvement is either specific or nonspecific.  Specific lesions manifest as noncaseatinggranulomas  that consist of mononuclear phagocytes, epithelioid macrophages and multinucleate giant cells  Whereas nonspecific lesions (EN) do not reveal granulomas on histopathologic examination.
    10. 10. Standard Treatment  A stepwise approach to patient care is appropriate  First Line: mild skin-limited disease.  Potent topical corticosteroids  clobetasol  Intralesionalinjections  triamcinolone(3-10 mg/mL)  First Line: deforming skin lesions or for widespread disease  Systemic therapy: prednisone 40-80 mg/day tapered used alone or in combination with antimalarials or methotrexate
    11. 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. 12. Treatment – Chronic/Refractory  TNF-αantagonists  Infliximab ( 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 cutaneoussarcoidosis, 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. 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 cutaneoussarcoidosis, and the clinical experience with tetracycline derivatives has been mixed.
    14. 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. 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. 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: Cutaneoussarcoidosis therapy updated.J Am AcadDermatol 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 cutaneoussarcoidosis: the role of thalidomide and other agents. Am J ClinDermatol. 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. 17. References cont.  James DG. Lupus pernio. Lupus. 1992 May;1(3):129-31  Fernandez-Faith E, McDonnell Cutaneoussarcoidosis: differential diagnosis. J.ClinDermatol. 2007 May-Jun;25(3):276-87.  Takashi Koyama, MD, Hiroyuki Ueda, MD, Kaori Togashi, MD, ShigeakiUmeoka, 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 CutaneousSarcoidosisSeminRespirCrit Care Med 2010; 31: 442-451  Doherty CB, Rosen T. Evidence-based therapy for cutaneoussarcoidosis. Drugs. 2008;68(10):1361-83.  PICS: TheAltitudeExperience.com, Dermis.net, dermatlas, Journal of Head and Neck Medicine, Lexic, emedicine, Access Medicine
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