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Marc A. Judson, MD, FCCP; Daniel Lackland, DrPH; William K. Mountford, PhD; Eleni Stagaki, MD
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Affiliations: Medical University of South Carolina Charleston, SC,  Sismangalo General Hospital Athens, Greece

Correspondence to: Marc A. Judson, MD, FCCP, Medical University of South Carolina, Pulmonary and Critical Care, CSB-812, 96 Jonathan Lucas St, Charleston, SC 29425; e-mail: judsonma@musc.edu


Dr. Judson has received research grants from Centocor, Celgene, Gilead Pharmaceuticals, and Merck. The other authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).


© 2009 American College of Chest Physicians


Chest. 2009;136(3):947. doi:10.1378/chest.09-1094
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To the Editor:

We appreciate the comments of Arias-Santiago and colleagues on our recently published article in CHEST (February 2009).1 We agree that there is confusion regarding various skin conditions that include the term “lupus” in their nomenclature. “Lupus pernio” (a form of cutaneous sarcoidosis), “lupus vulgaris” (a form of cutaneous tuberculosis), “lupus miliaris disseminata faciei” (a form of rosacea), and “chilblain lupus” are but a few examples.

Specifically, the authors expressed concern that chilblain lupus could be confused with lupus pernio. Although this is an obvious problem in terms of nomenclature, we feel that in clinical practice these entities are distinct and are unlikely to be confused for many of the reasons cited by Arias-Santiago and coauthors. Chilblain lupus is usually associated with systemic lupus erythematosus. It is extremely rare, with only 70 cases reported in a 2008 review.2 It involves primarily the toes and fingers; involvement of the ears or nose is rare.2 The lesions generally occur first during cold or damp periods. They are usually pruritic and later painful; such symptoms are extremely unusual with lupus pernio. Pathologically, chilblain lupus reveals vascular thrombosis and not granulomatous inflammation.2,3 Furthermore, although both systemic lupus erythematosus and sarcoidosis are systemic diseases, we disagree with Arias-Santiago and coauthors that they are difficult to distinguish clinically.

The problem with this nomenclature most probably stems from the word “pernio,” which refers to a localized inflammatory lesion of the skin resulting from an abnormal response to cold.4 Lupus pernio skin lesions have no relationship to cold exposure, but it is this unfortunate antiquated description that we believe has led to the confusion. It is probably more appropriate to refer to lupus pernio as “disfiguring facial sarcoidosis.” Nonetheless, we disagree that this confusion in nomenclature may lead to problems in distinguishing these entities clinically.

Stagaki E, Mountford WK, Lackland DT, et al. The treatment of lupus pernio: results of 116 treatment courses in 54 patients. Chest. 2009;135:468-476. [PubMed] [CrossRef]
 
Hedrich CM, Fiebig B, Hauck FH, et al. Chilblain lupus erythematosus: a review of literature. Clin Rheumatol. 2008;27:949-954. [PubMed]
 
Bouaziz JD, Barete S, Le Pelletier F, et al. Cutaneous lesions of the digits in systemic lupus erythematosus: 50 cases. Lupus. 2007;16:163-167. [PubMed]
 
Simon TD, Soep JB, Hollister JR. Pernio in pediatrics. Pediatrics. 2005;116:e472-e475. [PubMed]
 

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References

Stagaki E, Mountford WK, Lackland DT, et al. The treatment of lupus pernio: results of 116 treatment courses in 54 patients. Chest. 2009;135:468-476. [PubMed] [CrossRef]
 
Hedrich CM, Fiebig B, Hauck FH, et al. Chilblain lupus erythematosus: a review of literature. Clin Rheumatol. 2008;27:949-954. [PubMed]
 
Bouaziz JD, Barete S, Le Pelletier F, et al. Cutaneous lesions of the digits in systemic lupus erythematosus: 50 cases. Lupus. 2007;16:163-167. [PubMed]
 
Simon TD, Soep JB, Hollister JR. Pernio in pediatrics. Pediatrics. 2005;116:e472-e475. [PubMed]
 
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