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

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. 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
noncaseatinggranulomas
 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.
 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. 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-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. 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. 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: 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. 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