Lupus pernio
{{short description|Hardened facial lesions due to sarcoidosis}}
{{Infobox medical condition (new)
| name = Lupus pernio
| image = Lupus pernio 01.jpg
| caption = Cutaneous lesions of sarcoidosis (lupus pernio). Red-to-purple indurated plaques and nodules affecting the nose and cheeks.
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| field = Dermatology
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Lupus pernio is a chronic raised indurated (hardened) lesion of the skin, often purplish in color. It is seen on the nose, ears, cheeks, lips, and forehead. It is pathognomonic of sarcoidosis.{{cite book |author=James, William D. |author2=Berger, Timothy G.|title=Andrews' Diseases of the Skin: clinical Dermatology |publisher=Saunders Elsevier |year=2011 |isbn=978-1-4377-0314-6 |display-authors=etal}}{{rp|701}} The name "lupus pernio" is a misnomer, as microscopically this disease shows granulomatous infiltration and does not have features of either lupus nor pernio.{{cite book |author=Rapini, Ronald P. |author2=Bolognia, Jean L. |author3=Jorizzo, Joseph L. |title=Dermatology: 2-Volume Set |publisher=Mosby |location=St. Louis |year=2007 |isbn=978-1-4160-2999-1 }}
Signs and symptoms
The hallmarks of lupus pernio are violaceous or erythematous, indurated plaques that are mostly found on the cheeks and nose in the center of the face. Rarely, lesions may also affect the dorsum of the hands and feet and the ears.{{cite web | last1=Redissi | first1=Azza | last2=Penmetsa | first2=Gopi K. | last3=Litaiem | first3=Noureddine | title=Lupus Pernio | publisher=StatPearls Publishing | date=2023-07-10 | pmid=30725653 |url=https://www.ncbi.nlm.nih.gov/books/NBK536968/ | access-date=2024-02-24}} The symptoms of lupus pernio range from a few tiny nodules on the nose to vibrant plaques that cover both cheeks.{{cite journal | last=James | first=D. Geraint | title=Lupus Pernio | journal=Lupus | publisher=SAGE Publications | volume=1 | issue=3 | year=1992 | issn=0961-2033 | doi=10.1177/096120339200100302 | pages=129–131}} Lupus pernio lesions begin slowly but eventually penetrate and indurate into the underlying bone and cartilage, resulting in deformity. There is a higher chance of extracutaneous involvement, especially in the respiratory tract, in lupus pernio.{{cite journal | last1=SPITERI | first1=M.A. | last2=MATTHEY | first2=F. | last3=GORDON | first3=T. | last4=CARSTAIRS | first4=L.S. | last5=JAMES | first5=D.GERAINT | title=Lupus pernio: a clinico-radiological study of thirty-five cases | journal=British Journal of Dermatology | publisher=Oxford University Press (OUP) | volume=112 | issue=3 | year=1985 | issn=0007-0963 | doi=10.1111/j.1365-2133.1985.tb04859.x | pages=315–322| pmid=2983750 }}
Causes
The cause of cutaneous sarcoidosis is still not fully known. The generally acknowledged theory proposes that a complex interplay among genetic predisposition, immunological dysregulation, and environmental factors leads to sarcoidosis. It is believed that an augmented cell-mediated immune response to one or more undiscovered antigens is the cause of the granulomatous inflammation.{{cite journal | last1=Chen | first1=Edward S. | last2=Moller | first2=David R. | title=Etiology of Sarcoidosis | journal=Clinics in Chest Medicine | publisher=Elsevier BV | volume=29 | issue=3 | year=2008 | issn=0272-5231 | doi=10.1016/j.ccm.2008.03.011 | pages=365–377| pmid=18539232 }} Environmental exposure may have a role in the etiology of sarcoidosis, as evidenced by the development of granulomas that resemble sarcoid granulomas after exposure to metals such beryllium, zirconium, and aluminum. A number microorganisms have been suggested as potential etiologic agents of sarcoidosis, most notably mycobacteria and cutibacteria (previously propionibacteria).{{cite journal | last1=Chen | first1=Edward S. | last2=Moller | first2=David R. | title=Etiologies of Sarcoidosis | journal=Clinical Reviews in Allergy & Immunology | publisher=Springer Science and Business Media LLC | volume=49 | issue=1 | date=2015-03-15 | issn=1080-0549 | doi=10.1007/s12016-015-8481-z | pages=6–18}}
See also
References
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External links
{{Medical resources
| ICD10 = {{ICD10|D|86|3|d|80}} (ILDS D86.340)
| ICD9 = {{ICD9|135}}
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| Orphanet = 481662
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{{Sarcoidosis}}
Category:Monocyte- and macrophage-related cutaneous conditions
{{Cutaneous-condition-stub}}