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Lupus Pernio or Chilblain Lupus?: Two Different Entities FREE TO VIEW

Salvador A. Arias-Santiago, MD; María-Sierra Girón-Prieto, MD; José-Luis Callejas-Rubio, PhD; María-Antonia Fernández-Pugnaire, PhD; Norberto Ortego-Centeno, PhD
Author and Funding Information

San Cecilio University Hospital Granada, Spain

Correspondence to: Salvador A. Arias-Santiago, MD San Cecilio University Hospital, Av Dr. Oloriz 16, Granada, 18012 Spain; e-mail: salvadorarias@hotmail.es


The 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):946-947. doi:10.1378/chest.09-1005
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To the Editor:

In a recent issue of CHEST (February 2009), an interesting article1 was published about the treatment of lupus pernio in 54 patients. We would like to draw attention to the possible confusion that may exist, from a clinical point of view, between this type of cutaneous sarcoidosis and chilblain lupus (CL), which is a rare chronic form of cutaneous lupus erythematosus, defined by Hutchinson in 1888.2

Like lupus pernio, CL is characterized by erythematosus-purple plaques located in acral areas (most often, the nose and ears) but that are induced by exposure to cold or a drop in temperature (Fig 1), unlike other lesions of lupus erythematosus, which worsen with sun exposure, and sarcoidosis lesions, which remain unaltered with changes in temperature. CL can cause mild pain or itching, and can be associated with hyperhidrosis. During the evolution of the disease, the presence of atrophic scarring and residual pigmentation are common findings. Like others forms of lupus lesions, this is more common in women, and although it is sporadic, two families with autosomal-dominant inherited CL have been reported.

Figure Jump LinkFigure 1 A 48-year-old patient with CL lesion located in her nose induced by exposure to cold. The histopathologic study was compatible with lupus erythematosus. Subsequently she developed other lesions of chronic discoid lupus on the scalp, along with other clinical symptoms of systemic lupus erythematosus.Grahic Jump Location

The main difference with the cutaneous variety of sarcoidosis is the histopathologic study findings. In patients with lupus pernio, we observe granulomas without caseous necrosis but with few inflammatory infiltrates on the periphery; however, in patients with CL epidermal atrophy, the degeneration of the basal layer, periadnexal and perivascular inflammatory infiltrates are found, and other uncommon findings, such as dyskeratosis, increased mucin in the dermis, and the presence of granular deposits of Igs and complement in basement membrane, have been described.

To establish a proper diagnosis, Su et al3 suggested using the Mayo Clinic diagnostic criteria. These comprise two major criteria (skin lesions in acral locations induced by exposure to cold or a drop in temperature and evidence of lupus erythematosus in the skin lesions, as determined by histopathologic examination or indirect immunofluorescence study) and four minor criteria (coexistence of systemic lupus erythematosus or other skin lesion of discoid lupus erythematosus, response to anti-lupus therapy, and negative results of cryoglobulin and cold agglutinin studies). Both major criteria and one minor criterion need to be present to diagnose CL.

Recently, a treatment review of CL was conducted4 that stressed the importance of protection from cold by physical measures as a trigger factor and the use of topical or oral antibiotics if the lesions are infected. Therapy with topical corticosteroids or calcium channel blockers get a good response from patients. In patients with severe cases, systemic corticosteroids and mycophenolate may be used. However, according to these authors,4 therapy with antimalarial agents has a minor effect. Conversely, therapy with infliximab appears to be superior to therapy with systemic corticosteroids, with or without additional agents, for the treatment of lupus pernio.1 Instead, infliximab does not seem useful in the treatment of CL; even a case of CL induced by this drug has been described.5 In any case, it is essential to make a correct diagnosis; although clinically the two entities are similar and have in common their association with systemic disease (lupus erythematosus or sarcoidosis), the prognosis and treatment differ considerably.

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]
 
Hutchinson J. Harveian lectures on lupus: the varieties of common lupus. BMJ. 1888;1:58-63. [PubMed]
 
Su WP, Perniciaro C, Rogers RS III, et al. Chilblain lupus erythematosus (lupus pernio): clinical review of the Mayo Clinic experience and proposal of diagnostic criteria. Cutis. 1994;54:395-399. [PubMed]
 
Hedrich CM, Fiebig B, Hauck FH, et al. Chilblain lupus erythematosus: a review of literature. Clin Rheumatol. 2008;27:1341. [PubMed]
 
Richez C, Dumoulin C, Schaeverbeke T. Infliximab induced chilblain lupus in a patient with rheumatoid arthritis. J Rheumatol. 2005;32:760-761. [PubMed]
 

Figures

Figure Jump LinkFigure 1 A 48-year-old patient with CL lesion located in her nose induced by exposure to cold. The histopathologic study was compatible with lupus erythematosus. Subsequently she developed other lesions of chronic discoid lupus on the scalp, along with other clinical symptoms of systemic lupus erythematosus.Grahic Jump Location

Tables

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]
 
Hutchinson J. Harveian lectures on lupus: the varieties of common lupus. BMJ. 1888;1:58-63. [PubMed]
 
Su WP, Perniciaro C, Rogers RS III, et al. Chilblain lupus erythematosus (lupus pernio): clinical review of the Mayo Clinic experience and proposal of diagnostic criteria. Cutis. 1994;54:395-399. [PubMed]
 
Hedrich CM, Fiebig B, Hauck FH, et al. Chilblain lupus erythematosus: a review of literature. Clin Rheumatol. 2008;27:1341. [PubMed]
 
Richez C, Dumoulin C, Schaeverbeke T. Infliximab induced chilblain lupus in a patient with rheumatoid arthritis. J Rheumatol. 2005;32:760-761. [PubMed]
 
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