The Open Dermatology Journal




ISSN: 1874-3722 ― Volume 14, 2020
CASE REPORT

Isolated Linear Discoid Lupus Erythematosus of Scalp Following Lines of Blaschko



Abdurrahman Almurayshid1, *, Sulaiman Al Obaid2
1 Department of Medicine, College of Medicine, Prince Sattam Bin Abdulaziz University, Alkharj, Saudi Arabia
2 Department of Dermatology, Prince Mohammed Bin Abdulaziz Hospital, Riyadh, Saudi Arabia

Abstract

Discoid lupus erythematosus (DLE) is an inflammatory autoimmune disease leading to scarring and hair loss. Linear Discoid lupus erythematosus has been mentioned in the literature as a rare variant. This article describes isolated linear DLE of the scalp. We present a 26-year-old Saudi male who had unilateral linear DLE of the scalp with no involvement of other body sites and no systemic manifestations. This case is the first case of isolated linear DLE on the scalp. This presentation should be considered as a differential diagnosis of linear scarring alopecia as a distinct morphological pattern of DLE.

Keywords: Alopecia, Discoid lupus erythematosus, Scalp, Lichen planopilaris, Linear discoid lupus erythematosus, Hair Loss.


Article Information


Identifiers and Pagination:

Year: 2019
Volume: 13
First Page: 58
Last Page: 60
Publisher Id: TODJ-13-58
DOI: 10.2174/1874372201913010058

Article History:

Received Date: 27/08/2019
Revision Received Date: 12/11/2019
Acceptance Date: 16/11/2019
Electronic publication date: 20/12/2019
Collection year: 2019

Article Metrics:

CrossRef Citations:
0

Total Statistics:

Full-Text HTML Views: 437
Abstract HTML Views: 257
PDF Downloads: 160
ePub Downloads: 188
Total Views/Downloads: 1042

Unique Statistics:

Full-Text HTML Views: 239
Abstract HTML Views: 165
PDF Downloads: 111
ePub Downloads: 105
Total Views/Downloads: 620
Geographical View

© 2019 Almurayshid and Al Obaid.

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


* Address correspondence to this author at the Department of Medicine College of Medicine, Prince Sattam Bin Abdulaziz University, P.O. Box 173, Al-Kharj - 11942, Al-Kharj; Saudi Arabia; Tel: 00966115886101;
E-mail: almurayshid@gmail.com






1. INTRODUCTION

Discoid Lupus Erythematosus (DLE) is considered the most common form of chronic cutaneous lupus erythematosus. Cicatricial alopecia is the end stage of this inflammatory disease when it affects the scalp. Clinically, DLE of the scalp typically shows erythematous papules or plaques and keratotic follicular plugging associated with pigmentary changes and atrophy of the skin. It has a female predominance, especially in the fourth and fifth generations [1Fabbri P, Cardinali C, Giomi B, Caproni M. Cutaneous lupus erythematosus: Diagnosis and management. Am J Clin Dermatol 2003; 4(7): 449-65.
[http://dx.doi.org/10.2165/00128071-200304070-00002] [PMID: 12814335]
].

Discoid Lupus Erythematosus is usually localized to the head and neck in 80% of the cases and it usually affects sun-exposed areas. If lesions develop beyond the head and neck area it is termed disseminated DLE. [2Hassan M, Watters KA, Netchiporouk E. Linear discoid lupus erythematosus mimicking en coup de sabre morphea: A case report. SAGE Open Med Case Rep 2019.
[http://dx.doi.org/10.1177/2050313X19882843] [PMID: 31666954]
]. The risk of developing systemic lupus erythematosus in DLE patients is around 5-10%. Anti-nuclear antibodies can be seen in 20% of cases [3Somani N, Bergfeld WF. Cicatricial alopecia: Classification and histopathology. Dermatol Ther 2008; 21(4): 221-37.
[http://dx.doi.org/10.1111/j.1529-8019.2008.00203.x] [PMID: 18715291]
]. Cutaneous involvement of DLE along lines of Blaschko is quite rare [4Mao QX, Zhang WL, Wang Q, et al. Linear cutaneous lupus erythematosus/discoid lupus erythematosus in an adult. Postepy Dermatol Alergol 2017; 34(2): 177-9.
[http://dx.doi.org/10.5114/ada.2017.67086] [PMID: 28507499]
]. We present a very rare case of Linear DLE of the Scalp in lines of Blaschko.

2. CASE REPORT

A 26-year-old Saudi male presented to the dermatology clinic with a 10-month history of a skin lesion on the left temple area with progressive hair loss. The lesion was asymptomatic with no itching or redness. The patient denied any trauma to the scalp.

Physical examination showed a 1 × 12 cm unilateral linear well-defined pigmented plaque with atrophy, decreased hair density, perifollicular erythema and scaling over the superior border (Fig. 1A). There was no hair loss of the eyebrows, axillae, or groin area. The remaining skin, oral mucosa and nails were normal. He never complain of joint pain or photosensitivity and was otherwise healthy Dermoscopy showed areas of Perifollicular whitish halo, follicular keratotic plugs and telangiectasias (Fig. 1B) Antinuclear antibodies were negative.

Skin biopsy taken from the erythematous area showed the eccrine gland and interfollicular lymphocytic infiltrate involving the upper follicular epithelium with dense perifollicular fibrosis, atrophic follicles, thick basement membrane and subepidermal pigment incontinence. (Figs. 2A, 2B).

A diagnosis of linear DLE of the scalp was made. The patient was prescribed clobetasol solution in combination with triamcinolone acetonide injections which were (1ml; 10 mg/mL) administered monthly. After 4 sessions, the lesions improved, hair regrowth noticed and no new lesions appeared.

3. DISCUSSION

Linear Cutaneous lupus erythematosus rarely reported, especially DLE of the scalp. We found only 2 case reports on isolated DLE affecting the scalp [2Hassan M, Watters KA, Netchiporouk E. Linear discoid lupus erythematosus mimicking en coup de sabre morphea: A case report. SAGE Open Med Case Rep 2019.
[http://dx.doi.org/10.1177/2050313X19882843] [PMID: 31666954]
, 5Green 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. Pediatr Dermatol 1999; 16(2): 128-33.
[http://dx.doi.org/10.1046/j.1525-1470.1999.00030.x] [PMID: 10337677]
]. However, linear DLE was reported more frequently in other sites like the face, neck and trunk. Linear cutaneous lupus in the literature was also observed in the form of tumid lupus, Subacute cutaneous lupus erythematosus, scleroderma overlapping cutaneous lupus and lupus panniculitis [6Meena M, Balai M, Mehta S, Bharti G. Chronic cutaneous lupus wrythematosus in a blaschkoid pattern over face. Indian J Dermatol 2018; 63(2): 180-1.
[PMID: 29692464]
-8Seitz CS, Bröcker EB, Trautmann A. Linear variant of chronic cutaneous lupus erythematosus: A clue for the pathogenesis of chronic cutaneous lupus erythematosus? Lupus 2008; 17(12): 1136-9.
[http://dx.doi.org/10.1177/0961203308092425] [PMID: 19029283]
].

Fig. (1A)
Linear well-defined hyperpigmented atrophic patch with perifollicular scales.


Fig. (1B)
Dermoscopy showed Perifollicular whitish halo, follicular keratotic plugs and telangiectasias.


The patient did not have any history of trauma, and only one side of the scalp was affected. In contrast to this case, two similar case reports of linear DLE of the scalp had a facial extension [2Hassan M, Watters KA, Netchiporouk E. Linear discoid lupus erythematosus mimicking en coup de sabre morphea: A case report. SAGE Open Med Case Rep 2019.
[http://dx.doi.org/10.1177/2050313X19882843] [PMID: 31666954]
, 5Green 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. Pediatr Dermatol 1999; 16(2): 128-33.
[http://dx.doi.org/10.1046/j.1525-1470.1999.00030.x] [PMID: 10337677]
]. The patient had negative ANA and no other systemic manifestations of Lupus erythematosus as expected by majority with limited DLE [3Somani N, Bergfeld WF. Cicatricial alopecia: Classification and histopathology. Dermatol Ther 2008; 21(4): 221-37.
[http://dx.doi.org/10.1111/j.1529-8019.2008.00203.x] [PMID: 18715291]
].

Nevoid and Congenital skin diseases may follow lines of Blaschko. However, an inflammatory skin condition rarely has such a presentation [8Seitz CS, Bröcker EB, Trautmann A. Linear variant of chronic cutaneous lupus erythematosus: A clue for the pathogenesis of chronic cutaneous lupus erythematosus? Lupus 2008; 17(12): 1136-9.
[http://dx.doi.org/10.1177/0961203308092425] [PMID: 19029283]
]. Happle argued that in polygenic diseases, superimposed segmental manifestation should be used rather than segmental mosaicism. Type 1 or 2 segmental mosaicism is only applicable to monogenic inherited diseases or traits [9Happle R. Superimposed segmental manifestation of polygenic skin disorders. J Am Acad Dermatol 2007; 57(4): 690-9.
[http://dx.doi.org/10.1016/j.jaad.2007.06.039] [PMID: 17870433]
].

Lichen Planopilaris (LPP) should be considered as a differential diagnosis in this case. Differentiating DLE from LPP can be difficult as overlapping features occur clinically as well on pathology samples [10Nambudiri VE, Vleugels RA, Laga AC, Goldberg LJ. Clinicopathologic lessons in distinguishing cicatricial alopecia: 7 cases of lichen planopilaris misdiagnosed as discoid lupus. J Am Acad Dermatol 2014; 71(4): e135-8.
[http://dx.doi.org/10.1016/j.jaad.2014.04.052] [PMID: 25219732]
]. Clinical features favoring DLE include follicular plugging, depigmented or hypopigmented areas of skin and hyperpigmented surroundings. Although, histology of DLE may have perifollicular inflammation as seen in LPP, marked interstitial and perivascular inflammatory infiltration is often seen. Furthermore, thickening of the basement membrane and mucin deposition is observed [1Fabbri P, Cardinali C, Giomi B, Caproni M. Cutaneous lupus erythematosus: Diagnosis and management. Am J Clin Dermatol 2003; 4(7): 449-65.
[http://dx.doi.org/10.2165/00128071-200304070-00002] [PMID: 12814335]
, 3Somani N, Bergfeld WF. Cicatricial alopecia: Classification and histopathology. Dermatol Ther 2008; 21(4): 221-37.
[http://dx.doi.org/10.1111/j.1529-8019.2008.00203.x] [PMID: 18715291]
, 10Nambudiri VE, Vleugels RA, Laga AC, Goldberg LJ. Clinicopathologic lessons in distinguishing cicatricial alopecia: 7 cases of lichen planopilaris misdiagnosed as discoid lupus. J Am Acad Dermatol 2014; 71(4): e135-8.
[http://dx.doi.org/10.1016/j.jaad.2014.04.052] [PMID: 25219732]
].

Fig. (2A)
Dense eccrine gland and interfollicular lymphocytic infiltrate involving the upper follicular epithelium with concentric lamellar perifollicular fibrosis.


Fig. (2B)
PAS stain shows a positive outer root sheath (ORS) in the lower region of the hair follicle and a negative ORS in the upper region of the hair follicle.


The patient has liner DLE lesions involving only the scalp, making this the first case report presenting with isolated linear DLE exclusively on the scalp. We think that his lesions follow the lines of Blaschko. Unilateral affection and the absence of any trauma history make Kobnerization less likely. Linear DLE of the scalp should be considered as a differential diagnosis of linear scarring alopecia. This may be a distinct morphological pattern of DLE.

CONCLUSION

The first case of isolated linear DLE of the scalp is reported in this paper. When addressing linear scarring alopecia, linear DLE of the scalp must be recognized as a unique entity.

ETHICS APPROVAL AND CONSENT TO PARTIIPATE

This study was approved by the IRB (Institutional Review Board) (PSAU/COM/RC/IRB/A/35).

HUMAN AND ANIMAL RIGHTS

Not applicable.

CONSENT FOR PUBLICATION

Each participant signed an informed consent to participate in the study.

STANDARD OF REPORTING

CARE guidelines and methodology were followed to conduct the study.

FUNDING

None

CONFLICT OF INTEREST

The authors declare no conflict of interest, financial or otherwise.

ACKNOWLEDGEMENTS

Declared none.

REFERENCES

[1] Fabbri P, Cardinali C, Giomi B, Caproni M. Cutaneous lupus erythematosus: Diagnosis and management. Am J Clin Dermatol 2003; 4(7): 449-65.
[http://dx.doi.org/10.2165/00128071-200304070-00002] [PMID: 12814335]
[2] Hassan M, Watters KA, Netchiporouk E. Linear discoid lupus erythematosus mimicking en coup de sabre morphea: A case report. SAGE Open Med Case Rep 2019.
[http://dx.doi.org/10.1177/2050313X19882843] [PMID: 31666954]
[3] Somani N, Bergfeld WF. Cicatricial alopecia: Classification and histopathology. Dermatol Ther 2008; 21(4): 221-37.
[http://dx.doi.org/10.1111/j.1529-8019.2008.00203.x] [PMID: 18715291]
[4] Mao QX, Zhang WL, Wang Q, et al. Linear cutaneous lupus erythematosus/discoid lupus erythematosus in an adult. Postepy Dermatol Alergol 2017; 34(2): 177-9.
[http://dx.doi.org/10.5114/ada.2017.67086] [PMID: 28507499]
[5] 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. Pediatr Dermatol 1999; 16(2): 128-33.
[http://dx.doi.org/10.1046/j.1525-1470.1999.00030.x] [PMID: 10337677]
[6] Meena M, Balai M, Mehta S, Bharti G. Chronic cutaneous lupus wrythematosus in a blaschkoid pattern over face. Indian J Dermatol 2018; 63(2): 180-1.
[PMID: 29692464]
[7] Chen YA, Hsu CK, Lee JY, Yang CC. Linear lupus panniculitis of the scalp presenting as alopecia along Blaschko’s lines: A distinct variant of lupus panniculitis in East Asians? J Dermatol 2012; 39(4): 385-8.
[http://dx.doi.org/10.1111/j.1346-8138.2011.01455.x] [PMID: 22220763]
[8] Seitz CS, Bröcker EB, Trautmann A. Linear variant of chronic cutaneous lupus erythematosus: A clue for the pathogenesis of chronic cutaneous lupus erythematosus? Lupus 2008; 17(12): 1136-9.
[http://dx.doi.org/10.1177/0961203308092425] [PMID: 19029283]
[9] Happle R. Superimposed segmental manifestation of polygenic skin disorders. J Am Acad Dermatol 2007; 57(4): 690-9.
[http://dx.doi.org/10.1016/j.jaad.2007.06.039] [PMID: 17870433]
[10] Nambudiri VE, Vleugels RA, Laga AC, Goldberg LJ. Clinicopathologic lessons in distinguishing cicatricial alopecia: 7 cases of lichen planopilaris misdiagnosed as discoid lupus. J Am Acad Dermatol 2014; 71(4): e135-8.
[http://dx.doi.org/10.1016/j.jaad.2014.04.052] [PMID: 25219732]

Endorsements



"Open access will revolutionize 21st century knowledge work and accelerate the diffusion of ideas and evidence that support just in time learning and the evolution of thinking in a number of disciplines."


Daniel Pesut
(Indiana University School of Nursing, USA)

"It is important that students and researchers from all over the world can have easy access to relevant, high-standard and timely scientific information. This is exactly what Open Access Journals provide and this is the reason why I support this endeavor."


Jacques Descotes
(Centre Antipoison-Centre de Pharmacovigilance, France)

"Publishing research articles is the key for future scientific progress. Open Access publishing is therefore of utmost importance for wider dissemination of information, and will help serving the best interest of the scientific community."


Patrice Talaga
(UCB S.A., Belgium)

"Open access journals are a novel concept in the medical literature. They offer accessible information to a wide variety of individuals, including physicians, medical students, clinical investigators, and the general public. They are an outstanding source of medical and scientific information."


Jeffrey M. Weinberg
(St. Luke's-Roosevelt Hospital Center, USA)

"Open access journals are extremely useful for graduate students, investigators and all other interested persons to read important scientific articles and subscribe scientific journals. Indeed, the research articles span a wide range of area and of high quality. This is specially a must for researchers belonging to institutions with limited library facility and funding to subscribe scientific journals."


Debomoy K. Lahiri
(Indiana University School of Medicine, USA)

"Open access journals represent a major break-through in publishing. They provide easy access to the latest research on a wide variety of issues. Relevant and timely articles are made available in a fraction of the time taken by more conventional publishers. Articles are of uniformly high quality and written by the world's leading authorities."


Robert Looney
(Naval Postgraduate School, USA)

"Open access journals have transformed the way scientific data is published and disseminated: particularly, whilst ensuring a high quality standard and transparency in the editorial process, they have increased the access to the scientific literature by those researchers that have limited library support or that are working on small budgets."


Richard Reithinger
(Westat, USA)

"Not only do open access journals greatly improve the access to high quality information for scientists in the developing world, it also provides extra exposure for our papers."


J. Ferwerda
(University of Oxford, UK)

"Open Access 'Chemistry' Journals allow the dissemination of knowledge at your finger tips without paying for the scientific content."


Sean L. Kitson
(Almac Sciences, Northern Ireland)

"In principle, all scientific journals should have open access, as should be science itself. Open access journals are very helpful for students, researchers and the general public including people from institutions which do not have library or cannot afford to subscribe scientific journals. The articles are high standard and cover a wide area."


Hubert Wolterbeek
(Delft University of Technology, The Netherlands)

"The widest possible diffusion of information is critical for the advancement of science. In this perspective, open access journals are instrumental in fostering researches and achievements."


Alessandro Laviano
(Sapienza - University of Rome, Italy)

"Open access journals are very useful for all scientists as they can have quick information in the different fields of science."


Philippe Hernigou
(Paris University, France)

"There are many scientists who can not afford the rather expensive subscriptions to scientific journals. Open access journals offer a good alternative for free access to good quality scientific information."


Fidel Toldrá
(Instituto de Agroquimica y Tecnologia de Alimentos, Spain)

"Open access journals have become a fundamental tool for students, researchers, patients and the general public. Many people from institutions which do not have library or cannot afford to subscribe scientific journals benefit of them on a daily basis. The articles are among the best and cover most scientific areas."


M. Bendandi
(University Clinic of Navarre, Spain)

"These journals provide researchers with a platform for rapid, open access scientific communication. The articles are of high quality and broad scope."


Peter Chiba
(University of Vienna, Austria)

"Open access journals are probably one of the most important contributions to promote and diffuse science worldwide."


Jaime Sampaio
(University of Trás-os-Montes e Alto Douro, Portugal)

"Open access journals make up a new and rather revolutionary way to scientific publication. This option opens several quite interesting possibilities to disseminate openly and freely new knowledge and even to facilitate interpersonal communication among scientists."


Eduardo A. Castro
(INIFTA, Argentina)

"Open access journals are freely available online throughout the world, for you to read, download, copy, distribute, and use. The articles published in the open access journals are high quality and cover a wide range of fields."


Kenji Hashimoto
(Chiba University, Japan)

"Open Access journals offer an innovative and efficient way of publication for academics and professionals in a wide range of disciplines. The papers published are of high quality after rigorous peer review and they are Indexed in: major international databases. I read Open Access journals to keep abreast of the recent development in my field of study."


Daniel Shek
(Chinese University of Hong Kong, Hong Kong)

"It is a modern trend for publishers to establish open access journals. Researchers, faculty members, and students will be greatly benefited by the new journals of Bentham Science Publishers Ltd. in this category."


Jih Ru Hwu
(National Central University, Taiwan)


Browse Contents




Webmaster Contact: info@benthamopen.net
Copyright © 2020 Bentham Open