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Year : 2021  |  Volume : 12  |  Issue : 2  |  Page : 357-358  

An unusual case of linear cutaneous lupus erythematosus

1 Department of Dermatology, Katihar Medical College, Bihar, India
2 Department of Dermatology, KPC Medical College and Hospital, Kolkata, West Bengal, India
3 Department of Dermatology, University of Sydney, Australia

Date of Submission12-Jun-2020
Date of Decision19-Jul-2020
Date of Acceptance13-Sep-2020
Date of Web Publication22-Feb-2021

Correspondence Address:
Anupam Das
Building – “PRERANA”, 19, Phoolbagan, Kolkata - 700 086, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/idoj.IDOJ_465_20

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How to cite this article:
Kumar P, Das A, Raychaudhury T. An unusual case of linear cutaneous lupus erythematosus. Indian Dermatol Online J 2021;12:357-8

How to cite this URL:
Kumar P, Das A, Raychaudhury T. An unusual case of linear cutaneous lupus erythematosus. Indian Dermatol Online J [serial online] 2021 [cited 2022 Jan 27];12:357-8. Available from: https://www.idoj.in/text.asp?2021/12/2/357/308915


Linear cutaneous lupus erythematosus is an uncommon variant of lupus erythematosus (LE) presenting along the lines of Blaschko. Herein, we report a case of linear cutaneous lupus erythematosus, highlighting the necessity of biopsy to rule out clinical differentials in patients presenting with a linear arrangement of lesions on the face.

A 32-year-old lady presented with an asymptomatic, linear pigmented lesion on the forehead and left side of nose, of 2 months duration. The lesion started on the forehead and progressed caudally to attain the present status. There were no other symptoms including photosensitivity. Cutaneous examination revealed a linear hyperpigmented plaque extending from the left side of forehead to left ala nasi [Figure 1]. Upper part of the lesion (on forehead) was mildly atrophic and non-scaly. However, lower part of the lesion (over the nose) was slightly elevated and scaly. Rest of the mucocutaneous examination was unremarkable. Linear lichen planus and lichen planus pigmentosus were considered as differentials and a punch biopsy from the active part of the lesion over the nose was done. Histopathology showed prominent follicular plugging, lymphohistiocytic infiltration [Figure 2]a, and basal layer degeneration [Figure 2]b. The inflammation extended to interfollicular epidermis, causing focal basal layer vacuolar degeneration, pigment incontinence, and cytoid bodies. Histopathological findings were consistent with cutaneous lupus erythematosus. Linear lichen planus and linear lichen planus pigmentosus were ruled out, on the basis of prominent follicular plugging, periappendageal infiltration, and absence of hypergranulosis. Thus, on clinicopathological correlation, a diagnosis of linear cutaneous lupus erythematosus was made. Routine laboratory investigations including serum biochemistry panel were within normal limits. Antinuclear antibody (ANA) by immunofluorescence was weakly positive. Direct immunofluorescnce (DIF) from the lesion could not be done. The lady was treated with tacrolimus 0.1% ointment but was lost to follow up.
Figure 1: Linear, pigmented plaque extending from the forehead to left ala nasi. The upper part of the lesion is non-scaly and slightly atrophic. The nasal part of the lesion is slightly thickened and scaly

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Figure 2: (a) Follicular plugging, periappendageal infiltration (H and E ×40). (b) Lymphohistiocytic infiltration and basal layer vacuolar degeneration (H and E ×400)

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Cutaneous lupus erythematosus can present in varied morphologies and distribution patterns, linear being one of the rarest patterns. Linear discoid lupus erythematosus was first reported by Abe et al. in 1998.[1] It is mostly seen in children and young adults, without any gender predilection. Linear cutaneous lupus erythematosus may present with pigmented plaques, atrophic lesions, or verrucous plaques.[2],[3],[4],[5],[6] Such lesions have been reported to develop on the forehead, cheeks, nose, neck, chest, back, and upper extremities.[2],[3],[4],[5],[6],[7],[8] Photosensitivity and ANA are negative or weakly positive. Progression to systemic lupus erythematosus has not been reported, but as the condition is relatively new and little is known about the long-term prognosis, regular monitoring of the patient is required.[8]

The condition must be differentiated from clinical mimickers like linear lichen planus, lichen striatus, linear psoriasis, inflammatory linear verrucous epidermal nevus and linear morphea. Treatment options include topical anti-inflammatory agents like corticosteroids and calcineurin inhibitors; oral steroids, hydroxychloroquine, and other steroid-sparing immunosuppressives.[9]

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

Abe M, Ishikawa O, Miyachi Y. Linear cutaneous lupus erythematosus following the lines of Blaschko. Br J Dermatol 1998;139:307-10.  Back to cited text no. 1
Verma SB, Wollina U. Chronic disseminated discoid lupus erythematosus with linear lesions following Blaschko's lines on forearm and hand. J Dtsch Dermatol Ges 2012;10:129-30.  Back to cited text no. 2
Thind CK, Husain EA, Hewitt J. A rare linear atrophic lesion on the face. Clin Exp Dermatol 2009;34:e447-8.  Back to cited text no. 3
Bouzit N, Grézard P, Wolf F, Balme B, Perrot J. Linear cutaneous lupus erythematosus in an adult. Dermatology 1999;199:60-2.  Back to cited text no. 4
Green JJ, Baker DJ. Linear childhood discoid lupus erythematosus following the lines of Blaschko: A case report with review of the linear manifestations of lupus erythematosus. Ped Dermatol 1999;16:128-33.  Back to cited text no. 5
Abe M, Ohnishi K, Ishikawa O. Guess what? Linear cutaneouslupus erythematous (LCLE): Relationship with Blaschko's lines. Eur J Dermatol 2000;10:229-31.  Back to cited text no. 6
RequenaC, Torrelo A, De PI. Zambrano A. Linear childhood cutaneous lupus erythematosus following blaschko lines. J Eur Acad Dermatol Venereol 2002;16:618-20.  Back to cited text no. 7
Mao QX, Zhang WL, Wang Q, Xiao XM, Chen H, Shao XB, et al. Linear cutaneous lupus erythematosus/discoid lupus erythematosus in an adult. Postepy Dermatol Alergol 2017;34:177-9.  Back to cited text no. 8
Liu W, Vano-Galvan S, Liu JW, Qian YT, Fang K, Ma DL. Pigmented linear discoid lupus erythematosus following the lines of Blaschko: A retrospective study of a Chinese series. Indian J Dermatol Venereol Leprol 2020;86:359-65.  Back to cited text no. 9
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