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%.
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
Sarcoidosis is a
disease that can
affect any organ.
present in 20%-
35% of patients
Lupus pernio, first
described by Besnier in
1889, is a manifestation
of sarcoidal skin lesions.
Its name comes from Latin perniō = chilblain on the
Inflammation of the skin of the hands or feet, resulting
from exposure to cold
Thought to resemble a mild frostbite
Lupus Pernio Frostbite
More common in
African American’s and
Usually with long-
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.
Cutaneous Findings: violaceous, indurated plaques and
………………………… Dorsum of hand, fingers
…………………………………Cheeks and Lips
Cutaneous involvement is
either specific or nonspecific.
Specific lesions manifest as
that consist of mononuclear
macrophages and multinucleate
Whereas nonspecific lesions (EN)
do not reveal granulomas on
A stepwise approach to patient care is appropriate
First Line: mild skin-limited disease.
Potent topical corticosteroids
First Line: deforming skin lesions or for widespread
Systemic therapy: prednisone 40-80 mg/day tapered used
alone or in combination with antimalarials or methotrexate
Antimalarials and methotrexate may be used as monotherapy
for steroid-resistant sarcoidosis or in patients unable to
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
Treatment – Chronic/Refractory
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
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.
The course is usually
chronic, and severe
cosmetic disfigurement may
Lupus pernio, especially
involving the nasal rim, has
upper respiratory tract (50%)
•Rhinophyma when localized to the nose
•Malignant pleomorphic lymphoma
•Protracted superficial Wegener's granulomatosis
• Chensue SW, Warmington K, Ruth J, Lincoln P, Kuo MC, Kunkel SL: Cytokine
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manifestations of Sarcoidosis. The Journal of Musculoskeletal Medicine 2008,
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• 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
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Fernandez-Faith E, McDonnell Cutaneoussarcoidosis: differential diagnosis.
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Takashi Koyama, MD, Hiroyuki Ueda, MD, Kaori Togashi, MD, ShigeakiUmeoka,
MD, Masako Kataoka, MD and Sonoko Nagai, MD. Radiologic Manifestations of
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