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CORRESPONDENCE Table of Contents  
Ahead of print publication
Cutaneous annular erythema associated with Sjögren's syndrome successfully treated by baricitinib

1 Department of Dermatology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan
2 Department of Dermatology, Tri-Service General Hospital, National Defense Medical Center; Graduate Institute of Medical Sciences, National Defense Medical Center, Taipei, Taiwan
3 Department of Dermatology, Tri-Service General Hospital, National Defense Medical Center; Department of General Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan

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Date of Submission10-Mar-2022
Date of Decision06-Jun-2022
Date of Acceptance14-Jun-2022
Date of Web Publication09-Sep-2022

How to cite this URL:
Liu SW, Wang WM, Chiang CP, Hung CT, Chen YH. Cutaneous annular erythema associated with Sjögren's syndrome successfully treated by baricitinib. Dermatol Sin [Epub ahead of print] [cited 2022 Sep 29]. Available from: https://www.dermsinica.org/preprintarticle.asp?id=355702

Dear Editor,

Sjögren's syndrome (SS) is a chronic autoimmune disease that presents with symptoms of sicca and systemic involvement. Cutaneous manifestations are common extraglandular features, among which annular erythema is a distinct entity.

A 62-year-old Taiwanese woman had a 3-month history of multiple expanding annular lesions on her face and upper limbs. Seven months before appearance of the skin eruptions, she had been hospitalized for internal cerebral vein thrombosis and pulmonary embolism when protein C and protein S deficiency and SS (confirmed by high anti-Ro and anti-La titers, positive Schirmer's test, and sialoscintigraphy demonstrating salivary dysfunction) were diagnosed. She had been regularly taking warfarin, prednisolone (7.5 mg/day), leflunomide (20 mg/day), and cyclosporine (200 mg/day) for SS and its cytokine-related thrombophilia.

On physical examination, several indurated annular concentric plaques with a central purpuric hue were observed on both cheeks and dorsal arms [Figure 1]a; the patient permitted pictures of the lesions on the arms only], with the largest one on the right cheek measuring approximately 7 cm, causing disfigurement. Laboratory examination revealed the presence of rheumatoid factor and anti-Ro (937 U/ml), anti-La (1181 U/ml), and antinuclear antibodies (1:640).
Figure 1: (a) Several indurated annular concentric plaques with a central purpuric hue on the right dorsal arm (top) and the left dorsal arm (bottom left). Close-up view of the largest lesions on the right dorsal arm (bottom right). (b) Histological examination revealed perivascular and perieccrine lymphocytic infiltration, in addition to dermal mucin deposition (H and E, top, ×40; bottom, ×200). (c) Posttreatment skin lesions showed resolution with postinflammation hyperpigmentation (left: corresponding to [a] bottom left; right: corresponding to [a] bottom right).

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Skin biopsy of a lesion on the right arm showed perivascular and perieccrine lymphocytic infiltration in addition to dermal mucin deposition [Figure 1]b. Diagnosis of cutaneous lupus erythematosus was less likely due to the absence of vacuolar changes in the basal epidermis and the lack of immunoglobulin or complement deposition along the dermal–epidermal junction.

The patient was diagnosed with the annular erythema of the SS (AESS). She desired rapid resolution of the disfiguring lesions and strongly denied the use of chloroquine owing to the fear of hyperpigmentation it might induce. After a thorough discussion with the patient, she was treated with baricitinib (4 mg/day), which led to dramatic improvements in all skin lesions within 2 weeks [Figure 1]c. Therefore, baricitinib treatment was discontinued after 4 weeks. The clinical response was sustained throughout the 2-month follow-up period.

Skin involvement in SS manifests as vasculitic and nonvasculitic lesions.[1] Annular erythema, a distinct nonvasculitic manifestation of SS, is overwhelmingly associated with positive anti-Ro antibodies and preferentially affects but is not confined to the Asian populations only.[2] Three clinical types of AESS have been recognized: doughnut-ring-like erythema with a raised border mimicking Sweet disease (type I), subacute cutaneous lupus erythematosus (SCLE)-like polycyclic erythema with marginal scales (type II), and papular insect bite-like erythema (type III).[3] Type I clinical presentation was most fitting in our case.

The mechanisms that induce AESS involve infiltrating T cells that secrete interleukin (IL)-6, IL-16, interferon (IFN)γ, and transforming growth factor β and damage to epithelial cells via the FAS/FAS-L system.[3] Baricitinib acts through selective and reversible inhibition of JAK1 and JAK2, the downstream signaling pathway of IL-6 and IFNγ.[4]

Skin lesions in our patient developed and progressed during treatment with prednisolone, leflunomide, and cyclosporine. However, the patient showed a rapid and excellent response to baricitinib (4 mg) via its effect on blocking the IL-6 and IFNγpathways. To the best of our knowledge, this is the first report describing the successful treatment of AESS with baricitinib. However, a better interpretation of its efficacy requires a longer follow-up period and study of more cases in the future.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent form. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initial will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

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Conflicts of interest

Prof. Wei-Ming Wang, an editorial board member at Dermatologica Sinica, had no role in the peer review process of or decision to publish this article. The other authors declared no conflicts of interest in writing this paper.

  References Top

Roguedas AM, Misery L, Sassolas B, Le Masson G, Pennec YL, Youinou P. Cutaneous manifestations of primary Sjögren's syndrome are underestimated. Clin Exp Rheumatol 2004;22:632-6.  Back to cited text no. 1
Brito-Zerón P, Retamozo S, Akasbi M, Gandía M, Perez-De-Lis M, Soto-Cardenas MJ, et al. Annular erythema in primary Sjogren's syndrome: Description of 43 non-Asian cases. Lupus 2014;23:166-75.  Back to cited text no. 2
Katayama I, Kotobuki Y, Kiyohara E, Murota H. Annular erythema associated with Sjögren's syndrome: Review of the literature on the management and clinical analysis of skin lesions. Mod Rheumatol 2010;20:123-9.  Back to cited text no. 3
Furumoto Y, Gadina M. The arrival of JAK inhibitors: Advancing the treatment of immune and hematologic disorders. BioDrugs 2013;27:431-8.  Back to cited text no. 4

Correspondence Address:
Yi-Hsien Chen,
Department of Dermatology, Tri-Service General Hospital, National Defense Medical Center, No. 325, Sec. 2, Chenggong Road, Neihu District, Taipei 114
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1027-8117.355702


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