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CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 5  |  Page : 594-595
Co-occurrence of necrobiosis lipoidica, sarcoidosis and lichen planus


Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University, Yonago, Japan

Date of Web Publication29-Dec-2022

Correspondence Address:
Hiroyuki Goto
Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University, Yonago
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_1039_20

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How to cite this article:
Shiroma N, Goto H, Tani N, Sugita K, Yamamoto O. Co-occurrence of necrobiosis lipoidica, sarcoidosis and lichen planus. Indian J Dermatol 2022;67:594-5

How to cite this URL:
Shiroma N, Goto H, Tani N, Sugita K, Yamamoto O. Co-occurrence of necrobiosis lipoidica, sarcoidosis and lichen planus. Indian J Dermatol [serial online] 2022 [cited 2023 Feb 8];67:594-5. Available from: https://www.e-ijd.org/text.asp?2022/67/5/594/366090




Sir,

Sarcoidosis, lichen planus (LP), and necrobiosis lipoidica (NL) are relatively common types of dermatosis, and the coexistence of two of them—LP and NL or sarcoidosis and NL—has been reported.[1],[2] However, the co-occurrence of all of these three diseases has not been reported.

A 76-year-old woman was referred to our clinic with a complaint of red papules and plaques on her trunk and extremities. She was followed up and treated for diabetes mellitus, Sjögren syndrome, and autoimmune hepatitis. Physical examination revealed round, reddish-purple plaques, 2–3 cm in diameter, with central atrophy on her face and trunk [Figure 1]a. Yellowish plaques were also observed on her arm [Figure 1]b. On the inguinal region, there was a reddish-purple plaque, 5 cm in diameter, with a shiny and infiltrative rim [Figure 1]c. A blood test revealed an increased level of anti-nuclear antibody (×320, centromere pattern, normal: <×40), but levels of other antibodies associated with autoimmune disease, including anti-SS-A antibody, anti-SS-B antibody, and anti-double stranded DNA antibody, were normal. Skin biopsies were performed from lesions on the back, arm, and inguinal region. A biopsied specimen from the back revealed a stratiform pattern of alternatively arranged collagen degeneration and granuloma with inflammatory infiltrates [Figure 1]d. A specimen from the yellowish plaque on the arm showed granuloma with Langhans giant cells but no prominent infiltration of lymphocytes, namely naked-type granuloma with silica in the dermis [Figure 1]e. A specimen from the plaque on the inguinal lesion revealed lichenoid lymphocytic infiltration in the papillary dermis, vacuolization of the basal layer, and Civatte bodies and melanophages in the upper dermis [Figure 1]f. No mucin deposition was found by alcian-blue staining in any of the specimens (data not shown). Histopathological examination of the lesions of the back, arm, and groin showed NL, sarcoidosis, and LP, respectively. Immunohistochemistry revealed infiltration of CXCR3-positive lymphocytes, indicating Th1 lymphocytes, in all three lesions [Figure 1]g, [Figure 1]h, [Figure 1]i. The serum level of angiotensin-converting enzyme was 15.9 U/L (normal: 7.0–25.0 U/L). Because of the deterioration of autoimmune hepatitis, treatment with a corticosteroid was started and her cutaneous eruptions improved.
Figure 1: (a) Round, reddish-purple plaques with central atrophy on the back. (b) Yellowish plaques on the arm. (c) A reddish-purple plaque with a shiny and infiltrative rim on the inguinal region. (d) Histopathological picture of a plaque on the back showing collagen degeneration and granuloma (bar = 250 μm). (e) A histopathological picture of a plaque on the arm showing naked granuloma (bar = 100 μm). (f) Histopathology picture of an inguinal region showing lichenoid lymphocytic infiltration in the papillary dermis, vacuolization of the basal layer, and Civatte bodies and melanophages in the upper dermis (bar = 50 μm). (g) An immunohistopathological picture of anti-CXCR3 staining in necrobiosis lipoidica (bar = 100 μm, positive cells stained red). (h) An immunohistopathological picture of anti-CXCR3 staining in sarcoidosis (bar = 100 μm, positive cells stained red). (i) An immunohistopathological picture of anti-CXCR3 staining in lichen planus (bar = 100 μm, positive cells stained red).

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Sarcoidosis is a systemic granulomatous disease and is thought to be associated with a T-helper 1 (Th1) immune response.[3] Th1 lymphocytes and activated macrophages produce various cytokines and chemokines in mature granulomas. LP is also known as Th1-dominant inflammatory dermatosis, and Th1 lymphocytes play an important role in its pathogenesis.[4] However, the definite pathogenesis of NL has not been elucidated. Histopathologically, NL is characterized by a stratiform pattern of degenerated collagen and granuloma in the dermis to subcutis. Hassoun et al.[5] reported a case of NL that was successfully treated with ustekinumab. Ustekinumab targets IL-12/23p40 and suppresses IFN-γ production from Th1 lymphocytes. The effectiveness of ustekinumab for NL suggests that Th1 lymphocytes are associated with the pathogenesis of this disease. Therefore, Th1 lymphocytes may be involved in the pathogenesis of sarcoidosis, LP and NL, as shown in the figures. Activation of Th1 lymphocytes might have caused three different disorders as different phenotypes.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Lee J, Flowers RH, Cocks MM, Noland MB. Necrobiosis lipoidica associated with sarcoidosis. J Cutan Pathol 2018;45:944-8.  Back to cited text no. 1
    
2.
Balestri R, Rech G, Magnano M, Girardelli CR. Image Gallery: Never miss the details: The importance of dermoscopy in the differential diagnosis of coexistent inflammatory disorders. Br J Dermatol 2017;177:e2.  Back to cited text no. 2
    
3.
Ragusa F. Sarcoidosis and the Th1 chemokine MIG. Clin Ter 2018;169:e308-13.  Back to cited text no. 3
    
4.
Terlou A, Santegoets LA, van der Meijden WI, Heijmans-Antonissen C, Swagemakers SM, Spek PJ, et al. An autoimmune phenotype in vulvar lichen sclerosus and lichen planus: A Th1 response and high levels of microRNA-155. J Invest Dermatol 2012;132:658-66.  Back to cited text no. 4
    
5.
Hassoun LA, Sivamani RK, Sharon VR, Silverstein MA, Burrall BA, Tartar DM. Ustekinumab to target granulomatous dermatitis in recalcitrant ulcerative necrobiosis lipoidica: Case report and proposed mechanism. Dermatol Online J 2017;23:13030/qt3k32k916.  Back to cited text no. 5
    


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