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
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
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
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
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 - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
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
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
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
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
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
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
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
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
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
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
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 - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
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
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
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
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
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
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Observation Letter
89 (
4
); 585-588
doi:
10.25259/IJDVL_1299_20
pmid:
37067137

Bullous systemic lupus erythematosus in a pregnant woman with anaemia coexisting with asymptomatic hepatic haemangioma

Department of Dermatology, SVS Medical College, Mahbubnagar, Telangana, India
Corresponding author: Prof. Angoori Gnaneshwar Rao, F12 B8 HIG II APHB Baghlingampally, Hyderabad 500044, Telangana, India. dr_a_g_rao@yahoo.co.in
Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Rao AG, Naresh M, Sruthi Ch, Jhawar J. Bullous systemic lupus erythematosus in a pregnant woman with anaemia coexisting with asymptomatic hepatic haemangioma. Indian J Dermatol Venereol Leprol 2023;89:585–8.

Dear Editor,

Cutaneous manifestations have been reported in approximately 76% patients with systemic lupus erythematosus during the disease course and vesiculobullous lesions are encountered in less than 1% patients.1 Bullous systemic lupus erythematosus was first described by Pedro and Dahl in 1973. It is a distinctive clinical presentation of systemic lupus erythematosus; usually affecting young adult females and presenting with blisters on an erythematous base involving the face, trunk and extremities.

A 24-year-old female, in the second trimester of third pregnancy, presented to the department of dermatology of SVS Medical College with recurrent blisters and crusted erosions all over her body for last 5 months, associated with severe itching. Initially, she noticed blisters on her trunk which subsequently involved her face, upper and lower limbs and oral mucosa. The blisters ruptured with minimal trauma resulting in crusted erosions. She reported a history of concomitant low-grade fever and denied any history of similar skin rash in her previous two pregnancies or any history of photosensitivity, alopecia, and malar rash. Examination revealed multiple crusted plaques and erosions distributed bilaterally on the face [Figure 1], chest and upper back [Figure 2], both legs, lower abdomen and both buttocks. A single bulla was noted on the left upper chest [Figure 3]. We also noticed a single ulcer on her buccal mucosa extending over to the left angle of mouth. Nikolsky’s sign and Asboe Hansen’s sign were negative. The lesions appeared to heal with irregular hypopigmentation to depigmentation without atrophy, scar or milia. A provisional diagnosis of pemphigoid gestationis was made. Bullous systemic lupus erythematosus, linear immunoglobulin A (IgA) bullous dermatosis, and epidermolysis bullosa acquisita were considered as differentials. Investigations revealed anaemia (haemoglobin 9 gms/dL) normal reticulocyte count 1.8%, normal WBC count: 4700 cells/cubic millimeter and differential count: neutrophils: 65%, lymphocytes: 27%, eosinophils: 05%, monocytes: 03%, basophils: 0.0%. Peripheral blood smear demonstrared mildly microcytic, hypochromic, and anisocytic RBCs, and adequate platelets.

Figure 1:
Crust at left angle of mouth, both supra clavicular region and irregularly linear hypopigmented patches and macules on face and neck
Figure 2:
Multiple irregular depigmented patches and crusts on the back of the trunk
Figure 3:
Large bulla with pus on left clavicular region and crusted plaque on the left shoulder

Urine analysis revealed no abnormality and 24-hour urinary protein was 50 mg (normal < 150 mg). Liver function tests and renal function tests were unremarkable. The Coombs test was negative. Glucose–6–phosphate dehydrogenase was 10 U/g haemoglobin (normal range: 8.8–13.4U/g haemoglobin), venereal disease research laboratory test and human immunodeficiency virus 1 and 2 were unremarkable, and enzyme-linked immunosorbent assay test was done for pemphigoid gestationis antibody (BP180) and BP230 was negative. The antinuclear antibody test was positive with a speckled nuclear pattern. Anti-ds DNA (252 IU/mL) and anti-Sm antibodies were positive, while anti-Ro, anti-La, anti-U1RNP antibodies and anti-phospholipid antibodies were negative. Compliments 3 and 4 were normal. Antibodies to type VII collagen could not be assessed due to non-availability. Chest skiagram and 2-dimensional echocardiogram was unremarkable. Abdominal ultrasonography revealed a gravid uterus corresponding to 24 weeks of gestation and a single small haemangioma measuring 10 × 9 mm in the right lobe of liver. Tzanck smear showed neutrophils and was negative for acantholytic cells and multinucleated giant cells. Perilesional skin biopsy from the back demonstrated a subepidermal cleft with proteinaceous fluid, surrounded by neutrophils, few eosinophils and moderate lymphocytic infiltrate [Figure 4]. A direct immunofluorescence study showed dense immunoglobulin G (IgG) and sparse C3c and IgA deposits along the basement membrane [Figure 5]. To further characterize these immune deposits, an indirect immunofluorescence study was done on salt-split skin, which revealed dense IgG and sparse IgA deposits on the blister floor. [Figure 6]. She attained a score of 10 in the 2019 European League Against Rheumatism/(EULAR)classification criteria for diagnosis of systemic lupus erythematosus (fever-2, oral ulcers-2, anti-ds DNA antibodies and anti-Smith antibodies-6). Finally, she was diagnosed with bullous systemic lupus erythematosus type II (Camisa and Sharma criteria 1983) during pregnancy and we initiated dapsone 50 mg daily along with iron supplementation. Within a week, all lesions healed and we continued dapsone in a maintenance dose (25 mg/day). Ultrasound foetal monitoring was done every fortnight till 34 weeks and thereafter every week till delivery. Subsequently, she completed her pregnancy and delivered a healthy male baby; currently, he is nine months old, and there is no evidence of neonatal lupus erythematosus.

Figure 4:
Histopathology of skin; subepidermal cleft with proteinaceous material with neutrophils and eosinophils. Moderate infiltrate of lymphocytes, eosinophils and histiocytes in superficial dermis (H&E stain, ×400)
Figure 5:
Direct immunofluorescence study of biopsy from perilesional skin; strong IgG and weak IgA deposits along the basement membrane (FITC, ×200)
Figure 6:
Indirect immunofluorescence study on salt split skin; strong IgG and weak IgA deposits on the floor of the blister. (FITC, ×200)

The diagnosis of bullous systemic lupus erythematosus type II was established according to the criteria propounded by Camisa and Sharma.2 Gamman et al. have elucidated the role of immune complex-mediated inflammation in the pathogenesis of blister formation in bullous systemic lupus erythematosus. The pathogenic autoantibodies are directed against type VII collagen. It has been demonstrated in vitro studies that these antibodies activate complement and produce peptides that stimulate neutrophils and cause proteolysis resulting in dermo-epidermal junctional separation.3 Moreover, autoantigens such as the Sn RNP, Ro, La, Sm, nucleosomes, and ribosomes, with the help of toll-like receptors 7, induce inflammation in systemic lupus erythematosus by driving the production of IFN alpha, TNF alpha, IL-12 and other proinflammatory cytokines.4 The disease activity of systemic lupus erythematosus may be enhanced by pregnancy, and it may precipitate flares. Moreover, it has been reported that flares are highest during the second trimester of pregnancy and postpartum, which are attributed to lower levels of oestradiol and progesterone during the second trimester of pregnancy and decreased levels of steroid and elevated levels of prolactin during postpartum period.5 In consistence, the present patient experienced a flare with generalized bullous eruption during the second trimester of pregnancy. However, the patient did not manifest a flare during postpartum. Skin and joints are frequently involved during flares. Notably, bullous lesions in systemic lupus erythematosus are rare; only less than 5% of systemic lupus erythematosus patients develop bullous lesions. Moreover, the occurrence of bullous lesions in systemic lupus erythematosus is a pointer to the development of bullous systemic lupus erythematosus.6 Pregnancies in systemic lupus erythematosus are considered high-risk as they are prone to maternal and foetal complications. Smyth et al. conducted a recent meta-analysis involving 2571 pregnancies in systemic lupus erythematosus, and reported unsuccessful pregnancies in 23% and preterm births in 39%, while maternal complications reported were hypertension (16%), nephritis (16%), and pre-eclampsia (7%).7 There seems to be a correlation between blisters and renal involvement in bullous systemic lupus erythematosus, and most authors observed that the bullous eruption coincided with disease activity.8 Moreover, Huong et al. have reported renal involvement in 30.7% of systemic lupus erythematosus patients (n = 180).9 Nonetheless, there is no evidence of renal involvement in the present case. She is under observation for systemic involvement. Anaemia may be explained by iron deficiency. Additionally, autoantibodies in systemic lupus erythematosus might have caused impairment of erythropoietin production, leading to reduced iron metabolism and erythropoiesis.

Camisa and Sharma first reported the potential association between bullous systemic lupus erythematosus and glomerulonephritis.2 Furthermore, they reported a high incidence of IgA deposits along the basement membrane zone in patients with bullous systemic lupus erythematosus. However, there was no evidence of glomerulonephritis even though there were weak IgA deposits along the basement membrane zone in the present case.

The occurrence of bullous systemic lupus erythematosus in pregnancy is very rare, and so far, only three cases have been reported [Table 1].10-12 Interestingly, all three were known cases of systemic lupus erythematosus and developed bullous systemic lupus erythematosus in the first and second trimesters of pregnancy. Conversely, the present patient was diagnosed as bullous systemic lupus erythematosus without pre-existing systemic lupus erythematosus. Notably, all the reported cases developed systemic involvement during pregnancy, including nephritis, septicemia and central nervous system involvement, and were managed with systemic steroids, dapsone, azathioprine, hydroxychloroquine and broad-spectrum antibiotics, which resulted in favorable pregnancy outcome However, the present patient had no systemic involvement and was managed with only dapsone resulting in a successful outcome.

Table 1: Published case reports of bullous SLE in pregnancy
Authors Age of patient in years Duration of SLE Trimester System involved Treatment received Pregnancy outcome
Goncalves et al. (2006)10 25 2 years 2nd Renal
CNS
Prednisolone 40 mg/day Delivered healthy baby
Santos et al. (2013)11 25 1 year 1st Secondary bacterial infection Prednisolone 40 mg/day
Hydroxychloroquine
Broad spectrum antibiotics
Intra-uterine growth retardation
Vinicki et al. (2016)12 23 6 months 1st Renal Prednisolone 1 mg/kg/day
Azathioprine 2 mg/kg/day
Dapsone 100 mg/day
Postpartum details unavailable. Lost to follow up
Current report 24 Not known case of SLE 1st No Dapsone 100 mg/day Delivered healthy male baby

SLE: systemic lupus erythematosus

Bullous systemic lupus erythematosus usually does not respond to systemic steroids, unlike the other manifestations of systemic lupus erythematosus [Table 2]. Dapsone (25–50 mg daily) is the drug of choice in bullous systemic lupus erythematosus and it can arrest the development of blisters within 24–48 hours, but requires continuation for 6–12 months as there is a likelihood of relapse after discontinuation. The European League Against Rheumatism task force 2016 recommended certain drugs in the management of rheumatic diseases during pregnancy, such as antimalarials, azathioprine, cyclosporine, intravenous immunoglobulin, infliximab and etanercept, as these drugs are not associated with miscarriages and congenital malformations. Conversely, certain drugs such as cyclophosphamide, rituximab, methotrexate and tacrolimus are not recommended by the European League Against Rheumatism task force as they may promote miscarriages and congenital malformations.13

Table 2: Differences between bullous SLE in pregnancy and SLE in pregnancy
Bullous SLE SLE
Clinical features Vesiculobullous lesions often on the background of erythema or urticaria involving upper trunk, proximal extremities, neck and face.
Mucosal ulceration +
Photosensitive rash involving malar area of face.
Mucosal ulceration: ++
Diagnostic criteria Camisa and Sharma criteria (all 5 criteria) ARA criteria (4 or more)
Treatment Dapsone- drug of choice
No response to systemic steroids
Systemic steroid as indicated
Immunosuppressive drugs
Time for response to treatment Rapid response 24–48 hours longer
Risk to mother Low risk High risk
Risk to foetus Low risk High risk

SLE: systemic lupus erythematosus

In conclusion, bullous systemic lupus erythematosus in pregnancy poses a diagnostic and therapeutic challenge. It demands meticulous diligence for appropriate investigations and early therapy, as it affects both maternal and foetal health.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflict of interest

There are no conflicts of interest.

References

  1. , . Bullous eruption of systemic lupus erythematosus In: , , eds. Management of Blistering Diseases. London: Chapman and Hall Ltd; . p. :263-75.
    [Google Scholar]
  2. , . Vesiculobullous systemic lupus erythematosus. Report of two cases and a review of the literature. J Am Acad Dermatol. 1983;9:924-33.
    [CrossRef] [PubMed] [Google Scholar]
  3. , , , , . Evidence supporting a role for immune complex-mediated inflammation in the pathogenesis of bullous lesions of systemic lupus erythematosus. J Invest Dermatol. 1983;81:320-5.
    [CrossRef] [PubMed] [Google Scholar]
  4. , , , , . Mechanism of auto antibody production in systemic lupus erythematosus. Front Immunol. 2015;6:228.
    [CrossRef] [PubMed] [Google Scholar]
  5. , , . Challenges of lupus pregnancies. Rheumatology (Oxford). 2008;47(Suppl):9-12. iii
    [CrossRef] [PubMed] [Google Scholar]
  6. , , , , . Bullous lupus: An unusual initial presentation of systemic lupus erythematosus in an adolescent girl. Pediatr Dermatol. 2010;4:373-6.
    [CrossRef] [PubMed] [Google Scholar]
  7. , , , , , . A systematic review and meta-analysis of pregnancy outcomes in patients with systemic lupus erythematosus and lupus nephritis. Clin J Am Soc Nephrol. 2010;5:2060-8.
    [CrossRef] [PubMed] [Google Scholar]
  8. , , , , , . Concomitant lupus nephritis and bullous eruption in systemic lupus erythematosus. Nephrol Dial Transplant. 1999;14:1739-43.
    [CrossRef] [PubMed] [Google Scholar]
  9. , , , , , , et al. Renal involvement in systemic lupus erythematosus. A study of 180 patients from a single center. Medicine (Baltimore). 1999;78:148-66.
    [CrossRef] [PubMed] [Google Scholar]
  10. , , , , , , et al. Bullous systemic lupus erythematosus: A case report. FC–08–006. Abstracts of the European Congress of Perinatal and Neonatal Medicine 2006
    [Google Scholar]
  11. , , , , , . Bullous systemic lupus erythematosus in a pregnant woman: A case report. Rev Bras Rheumatol. 2013;53:438-40.
    [PubMed] [Google Scholar]
  12. , , , , , , et al. Bullous systemic lupus erythematosus in a pregnant woman associated with lupus nephritis responding to dapsone. J Rheumatol Arthritic Dis. 2016;1:1-3.
    [Google Scholar]
  13. , , , , , , et al. Steroid hormones and disease activity during pregnancy in systemic lupus erythematosus. Arthritis Rheum. 2002;47:202-9.
    [CrossRef] [PubMed] [Google Scholar]

Fulltext Views
6,493

PDF downloads
33,545
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections