Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
Therapy Letters
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Letter to the Editor - Observation Letter
doi: 10.4103/ijdvl.IJDVL_603_18
PMID: 32461382

Generalized facial pigmentation: An uncommon presentation of cutaneous lupus erythematosus

Alba Calleja Algarra1 , Raquel Aragón Miguel1 , Marta Prieto Barrios1 , Rafael Llamas Martín1 , Carlos Zarco Olivo1 , Maria Del Rosario Haro Ramos2 , Jose Luis Rodríguez Peralto3 , Pablo Luis Ortiz Romero1 , Concepcion Postigo Llorente1
1 Department of Dermatology, Institute I + 12, Universitary Hospital 12 de Octubre, Complutense University, Madrid, Spain
2 Department of Dermatology, Hospital Fundación Jiménez Díaz, Madrid, Spain
3 Department of Anatomopathology, Universitary Hospital 12 de Octubre, Madrid, Spain

Correspondence Address:
Alba Calleja Algarra
Department of Dermatology, Universitary Hospital 12 de Octubre, Avenida de Córdoba, S/N, 28041 Madrid
Published: 21-May-2020
How to cite this article:
Calleja Algarra A, Aragón Miguel R, Prieto Barrios M, Llamas Martín R, Zarco Olivo C, Haro Ramos MR, Rodríguez Peralto JL, Ortiz Romero PL, Llorente CP. Generalized facial pigmentation: An uncommon presentation of cutaneous lupus erythematosus. Indian J Dermatol Venereol Leprol 2020;86:431-435
Copyright: (C)2020 Indian Journal of Dermatology, Venereology, and Leprology


Post-inflammatory hyperpigmentation occurs when the basement membrane of the skin is damaged and this is seen in many inflammatory skin diseases such as lupus erythematosus. It appears in the late phase of many skin lesions and hampers the identification of specific histological features which are characteristic of the triggering disease.[1]

Post-inflammatory pigmentation is widely seen in the periphery of the skin lesions of discoid lupus erythematosus or in the late phase of acute cutaneous lupus erythematosus. However, cutaneous lupus erythematosus presenting predominantly with macular pigmentation is uncommon and often misdiagnosed.[2],[3]

We present six patients who came with diffuse cutaneous facial pigmentation, as the main manifestation of cutaneous lupus erythematosus, showing histological features of cutaneous lupus.

Our patients included four men and two women from Hospital 12 de Octubre and Hospital Fundación Jiménez Díaz (Madrid, Spain) with an average age of 51.33 years and medium-dark phototypes (III-V). Time until diagnosis ranged from 3 months to 10 years.

Clinically, they showed a diffuse, non well-demarcated grey-brown pigmentation, mainly on the forehead, temples and cheeks [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]. Patient no. 1 showed involvement of the ear lobes and patient no. 4 had pigmentation in the V area of the neck as well. Five cases showed concomitant facial erythema, although pigmentation was the predominant feature in all of them. In patient no. 4, who had features of systemic lupus erythematosus, the worsening of pigmentation was observed concurrently with disease outbreaks and the onset of other general manifestations, particularly joint-related symptoms. Besides, this patient had subcutaneous nodules in buttocks and thighs concurrently with one of the outbreaks of the systemic disease. Lupus panniculitis was suspected; however, the biopsy did not show any specific finding. All the patients showed positive autoimmunity markers [Table - 1].

Figure 1: Patient 1 with diffuse slate-grey pigmentation on face with mild erythema
Figure 2: Patient 2 with brownish pigmentation on the forehead with marked erythema
Figure 3:
Figure 4: Patient 6 with brownish pigmentation limited to malar and mandibular area with marked skin erythema
Table 1: Clinical and serological features of the patients

In all cases, drug-induced skin pigmentation was ruled out and epicutaneous patch tests were performed with GEIDAC's (Spanish Research Group on Contact Dermatitis and Cutaneous Allergy) standard battery which showed negative results.

The area of skin which showed maximum pigmentation was selected for skin biopsy. Histological examination was carried out in all cases and it revealed interface dermatitis with vacuolar degeneration of the basal cells, a dense lympho-mononuclear perivascular and perifollicular infiltrate with abundant melanophages in the papillary and high reticular dermis. The colloidal iron technique revealed mucin deposits in the dermis in all cases. This was compatible with pigmented lupus erythematosus [Figure - 5] and [Figure - 6]. More than one biopsy was needed to reach the diagnosis in three of the patients. Due to the nonavailability of the direct immunofluorescence technique at our hospital, this procedure was not performed. Each of the patient received hydroxychloroquine at a dose of 400 mg daily, together with strict photoprotection. Pigmentation on the forehead of patient no. 1 improved with hydroxychloroquine and strict photoprotection by wearing a hat. Pigmentation also improved in patient no. 3, but in rest of the patients pigmentation remained unchanged. Patient no. 4 had a recalcitrant clinical course which was controlled only with intravenous immunoglobulin infusions. She received 16 monthly cycles of intravenous immunoglobulin at a dose of 400 mg/kg/day for 5 consecutive days, achieving non-pregression of the facial pigmentation [Figure - 3]b and [Figure - 3]b.

Figure 5: Patient 1: Histopathological examination showing vacuolar interface dermatitis with dense lympho-mononuclear perivascular and perifollicular infiltrate and melanin incontinence (H and E, ×40)
Figure 6: Patient 4: Histopathological examination showing focal interface dermatitis and superficial perivascular lymphocytic infiltrate in papillary dermis with melanin deposits (H and E, ×400)

Although not often recognized, cutaneous lupus erythematosus may present itself with diffuse facial pigmentation as the predominant disease manifestation. Boyd reported eleven patients who presented with solitary pigmented macules with histological features of discoid lupus erythematosus. These authors conclude that this peculiar presentation occurs in older patients and shows a good response to topical corticosteroids.[4] Later, Taddio et al. reported the case of a 14-year-old girl with diffuse facial pigmentation showing histological characteristics of cutaneous lupus erythematosus in the context of a first systemic disease outbreak which was resolved with specific treatment.[5]

Recently, Khullar et al. reported three patients with generalized brownish-grey facial pigmentation with a diagnosis of pigmented lupus erythematosus.[6]

Reaching this diagnosis can be tricky and other causes of post-inflammatory pigmentation such as Riehl's melanosis, lichen planus pigmentosus and drug-related pigmentation should be included in the differential diagnosis. It is especially relevant to exclude hydroxychloroquine-associated pigmentation since it can lead to discontinuation of a beneficial treatment. Histopathological examination is the key and would show granular deposits of yellow-brown pigment and no inflammation.

We believe our patients have a true form of cutaneous lupus erythematosus whose main dermatological manifestation is pigmentation. Our series suggests that this feature is more common among patients with medium-dark phototypes and being the predominant skin manifestation it could be a marker of cutaneous lupus activity in such patients. There is a great clinical variability in the degree and distribution of pigmentation. The most common pigmentation pattern is a spotted or reticular brown pigmentation on the forehead, temples and malar areas. However, sometimes the lesions have a scattered pattern. This heterogeneity makes the patient's clinical context and skin biopsy essential for diagnosis.

In conclusion, when we come across a patient with generalized facial pigmentation, pigmented lupus erythematosus should always be consdered and histological evaluation should be done to confirm this diagnosis. Dermatologists should be aware of this possibility since it can be the only feature of lupus erythematosus.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for 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


Conflicts of interest

There are no conflicts of interest.

Hejazi EZ, Werth VP. Cutaneous lupus erythematosus: An update on pathogenesis, diagnosis and treatment. Am J Clin Dermatol 2016;17:135.
[Google Scholar]
Walling HW, Sontheimer RD. Cutaneous lupus erythematosus: Issues in diagnosis and treatment. Am J Clin Dermatol 2009;10:365-81.
[Google Scholar]
Obermoser G, Sontheimer RD, Zelger B. Overview of common, rare and atypical manifestations of cutaneous lupus erythematosus and histopathological correlates. Lupus 2010;19:1050-70.
[Google Scholar]
Boyd AS. Localized chronic cutaneous lupus erythematosus masquerading as pigmented lesions: A new clinical subset? Lupus 2006;15:292-5.
[Google Scholar]
Taddio A, Franzil A, Lepore L. Postinflammatory hyperpigmentation (PIH): The presenting sign of systemic lupus erythematosus. Clin Exp Rheumatol 2008;26:156.
[Google Scholar]
Khullar G, De D, Narang T, Saikia UN, Saikia B, Handa S. Pigmented macular variant of chronic cutaneous lupus erythematosus: An under-recognized subset in dark skin. Clin Exp Dermatol 2017;42:793-5.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections