Lupus pernio D86.34

Last updated on: 29.10.2020

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Synonym(s)

Large Nodular Sarcoidosis

History
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Besnier, 1889

Definition
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Rare, plaque or nodular form of sarcoidosis with blue-red, frostbitten 3.0-5.0 cm plaques or nodules on cheeks, earlobes and nose.

Clinical features
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On cheeks, nose, forehead and earlobes up to 5.0 cm in size, blurred, round or oval, deep red plaques, reddish-blue plaques at lower outside temperatures or slightly elevated, surface-smooth firm knots. No significant subjective symptoms. The surface structure may change irregularly like a pavement during longer periods of time.

Furthermore, there is a risk of ulceration of the lesions if they remain in place for many years, which can lead to nasal septum defects and associated instability of the cartilaginous nasal skeleton if they are located on the nose and upper lip.

Therapy
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S.u. Sarcoidosis.

Progression/forecast
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There are reports that this form of sarcoidosis is more often associated with sarcoidosis of internal organs, especially the upper respiratory tract and lungs (monitoring sign).

Note(s)
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Remark: In our opinion the term "Lupus pernio" has only historical meaning. It is suggested to use this term only in parentheses in a diagnostic table, especially since for large-nodular facial involvement a syndromal peculiarity compared to other large-nodular sarcoidosis localizations is not assured (Anjaneyan G et al. 2013).

Literature
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  1. Anjaneyan G et al (2013) Lupus pernio without systemic involvement. Indian Dermatol Online J 4:314-317.
  2. Besnier E (1889) Lupus pernio de la face; synovites fongueuses (scrofulo-tuberculeuses) symétriques des extrémités superieures. Annales de dermatologie et de syphilographie (Paris) 2nd series; 10: 333-336
  3. Ishak R et al (2015) Cutaneous sarcoidosis: clinicopathologic
  4. study of 76 patients from Lebanon. Int J Dermatol 54:33-41
  5. Mizuno K et al (2015) Lupus pernio with 2 years of preceding symptomatic gastric sarcoidosis. J Dermatol 42:330-331
  6. Shah A et al (2014) Lupus pernio--a patient case study. J Vis Commun Med 37(1-2):19-23
  7. Vashisht D et al (2014) Lupus pernio. Med J Armed Forces India 70:281-283

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020