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Table of Contents
Year : 2021  |  Volume : 12  |  Issue : 1  |  Page : 42-43

Chilblain lupus and discoid rashes in systematic lupus erythematosus

1 Saint Louis University Hospital of the Sacred Heart-Baguio City, College of Medicine-Virgen Milagrosa University Foundation,San Carlos City, Pangasinan, Philippines, Asia
2 Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Date of Submission07-Oct-2020
Date of Acceptance08-Oct-2020
Date of Web Publication06-Jan-2021

Correspondence Address:
Dr. Latika Gupta
Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/INJMS.INJMS_124_20

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How to cite this article:
Gupta P, Gupta L. Chilblain lupus and discoid rashes in systematic lupus erythematosus. Indian J Med Spec 2021;12:42-3

How to cite this URL:
Gupta P, Gupta L. Chilblain lupus and discoid rashes in systematic lupus erythematosus. Indian J Med Spec [serial online] 2021 [cited 2023 May 30];12:42-3. Available from: http://www.ijms.in/text.asp?2021/12/1/42/306248

Dear Editor,

A 32-year-old postpartum female presented with low-grade fever associated with inflammatory arthralgias of the small joints of hands for 2 months, which began 6 months post delivery. She also noted recent discoloration of the toes. On physical examination, dusky discoloration of the 1st and 2nd digits of the right foot [Figure 1]a was noted, which did not blanch on pressure. Further examination revealed hyperpigmented patches on the right concha with fine scales interspersed with areas of depigmentation, suggestive of discoid lupus erythematosus (DLE) [Figure 1]b. She also had a painless oral ulcer measuring 1 cm×1 cm with a necrotic base on the hard palate. The patient developed blue discoloration of the fingers on exposure to cold environment of the clinic [Figure 1]c. She confirmed that these are painful, and turn red upon rewarming, suggesting Raynaud’s phenomenon (RP). Rest of the systematic and musculoskeletal examination was unremarkable. Fever and inflammatory arthralgia in a young postpartum woman with chilblain lupus, DLE, and RP led to a suspicion of systemic lupus erythematosus (SLE).
Figure 1: Clinical manifestations: (a) Chilblain lupus erythematosus on the toes. (b) Hyperpigmentation with scarring in the ear concha. (c) Raynaud phenomenon involving the fingers

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  Discussion Top

Fever can be the presenting symptom in 30%–80% of active SLE. Arthralgias are more commonly described, in nearly 90% of cases.[1] Arthritis in lupus can be mild and is usually nonerosive, with normal joints on examination. This patient did not exhibit the tell-tale cutaneous signs of active SLE, such as malar rash or annular psoriasiform rashes of subacute cutaneous lupus. However, a careful clinical examination revealed other signs that the patient was not aware of.

Chilblain lupus erythematosus (chilblain LE) is a nonspecific cutaneous lesion, which is reported in 25% of SLE. Chilblain LE presents with tender, bright red to reddish-blue papules, nodules, or plaques on the toes, fingers, nose, or ears, precipitated by cold exposure. Chilblain LE is a type of chronic cutaneous LE, is autoimmune in nature, and is mostly sporadic. Familial forms have been described, especially in children. It is important to distinguish these from the reddish-blue rashes of idiopathic chilblains and lupus pernio. Idiopathic chilblains are due to dermal blood flow disruption due to intense cold exposure. Although the histologic findings are similar, immunofluorescence does not exhibit immune deposits. Lupus pernio, seen on the face and ears, is a cutaneous feature of sarcoidosis, with an association with upper respiratory disease and African-American/Hispanic population. On histopathology, it can be distinguished by the presence of non-caseating granulomas.[2]

Although only 5% of DLE have SLE, DLE lesions may be present in 15%–30% of SLE. The discoid shape with adherent keratotic scaling and follicular plugging is a characteristic feature. Atrophic scarring is also present in these lesions that predominantly occur on the face and scalp.[3] The occurrence of DLE on the ears (particularly conchal bowls) may be missed, and should be actively looked for in all cases. Painless palatal ulcers are also highly specific for a diagnosis of SLE.[3]

RP, although not classic of SLE, reportedly occurs in 15%–30% of cases.[1] It is a result of a vasospastic response to cold temperatures or emotional stress. This results in the affected part turning white and then blue. As blood flow returns, the area turns red.[3] The occurrence of RP may be a salient marker of an underlying connective tissue disease (CTD). Nailfold capillaroscopy (NFC) may reveal peripheral microangiopathy of active vascular dilatation that is different from capillary dropouts seen in other CTDs such as systemic sclerosis or inflammatory myositis.[4],[5] Moreover, abnormalities on NFC may reflect the extent of microvascular involvement in SLE, and differ in those with cutaneous disease versus those without.[6]

Thus, cutaneous signs may be characteristic and the key to clinch a diagnosis of lupus in the clinic. Lesions on the oral mucosa, ears, and toes should be actively sought for timely diagnosis and management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD. Manifestations of systemic lupus erythematosus. Maedica (Bucur) 2011;6:330-6.  Back to cited text no. 1
Patel S, Hardo F. Chilblain lupus erythematosus. BMJ Case Reports 2013; 2013:bcr2013201165.  Back to cited text no. 2
Uva L, Miguel D, Pinheiro C, Freitas JP, Gomes MM, Filipe P. Cutaneous manifestations of systemic lupus erythematosus. AQ3: Autoimmune Dis. 2012;2012:834291. doi: 10.1155/2012/834291. Epub 2012 Jul 25. PMID: 22888407; PMCID: PMC3410306.  Back to cited text no. 3
Ragab O, Shmawy A, Abdo M, Mokbel A. Nailfold capillaroscopy in systemic lupus erythematosus. Egypt Rheumatologist 2011;33:61-7. [doi: 10.1016/j. ejr. 2010.12.003.  Back to cited text no. 4
Shenavandeh S, Habibi S, Habibi Y, Nazarinia M. Mechanic hands: Clinical and capillaroscopy manifestations of patients with connective tissue diseases presented with and without mechanic hands. Clin Rheumatol 2019;38:2309-18.  Back to cited text no. 5
Moneib HA, Salem SA, Aly DG, Khedr HT, Wafaey HA, Hassan HE. Assessment of serum vascular endothelial growth factor and nail fold capillaroscopy changes in systemic lupus erythematosus with and without cutaneous manifestations. J Dermatol. 2012;39:52-7. [doi: 10.1111/j. 1346-8138.2011.01322.x].  Back to cited text no. 6


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