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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2017  |  Volume : 28  |  Issue : 3  |  Page : 653-656
Unusual presentation of systemic lupus erythematosus

Department of Nephrology, M. S. Ramaiah Memorial Hospital, Bengaluru, Karnataka, India

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Date of Web Publication18-May-2017


Bullous systemic lupus erythematosus is a rare distinctive subepidermal blistering disorder that can occur in patients with systemic lupus erythematosus (SLE). It is histologically characterized by a neutrophil-predominant infiltrate in the subepidermal region with deposition of immunoglobulins (IgG, IgA, IgM), C3, and auto-antibodies against collagen VII. Herein, we report a case of a 13-year-old girl who presented with bullous SLE and Class III lupus nephritis, which is extremely rare at this age.

How to cite this article:
Mahesh E, Madhyastha PR, Varma V, Gurudev K C, Gireesh M S, Bande SR. Unusual presentation of systemic lupus erythematosus. Saudi J Kidney Dis Transpl 2017;28:653-6

How to cite this URL:
Mahesh E, Madhyastha PR, Varma V, Gurudev K C, Gireesh M S, Bande SR. Unusual presentation of systemic lupus erythematosus. Saudi J Kidney Dis Transpl [serial online] 2017 [cited 2020 May 31];28:653-6. Available from: http://www.sjkdt.org/text.asp?2017/28/3/653/206468

   Introduction Top

Bullous systemic lupus erythematosus (BSLE) is an auto-antibody-mediated subepidermal blistering disease that occurs in patients with systemic lupus erythematosus (SLE).[1] Pedro and Dahl described the first case of BSLE in 1973. The association of BSLE with lupus nephritis (LN) has been reported in adults.[2] To our knowledge, only five cases of BSLE with LN have been reported in children.

   Case Report Top

A 13-year-old female was admitted to M.S. Ramaiah Memorial Hospital in December 2013 with a history of blisters over the palms, soles, and few erosions over the neck, face, and oral cavity of one-week duration.

Clinical examination revealed multiple fluid- filled bullae present all over the body, palms, and soles with few erosions present around the neck, face, and oral cavity [Figure 1] and [Figure 2]. Curdy white discharge was seen over the oral ulcers. The ano-genital regions were spared. The bullae on the sun-exposed areas had an erythematous base while those on the abdomen and legs had a clear base. She did not have hair loss, Raynaud’s phenomenon, digital tip ulcers, chest pain, cough, hemoptysis, seizure, or psychosis. She had no history of blistering lesions in the past, and there was no history of similar lesions in her family.
Figure 1: Bullous lesions seen on the palms.

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Figure 2: Bullous lesions seen on the soles.

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On examination, she weighed 38 kg with height of 152 cm and body surface area of 1.29 m2. She was pale, with pulse rate of 108/min and blood pressure of 100/70 mm Hg.

She was empirically treated with intravenous (i.v.) ceftriaxone and other supportive measures. Urine showed proteinuria and active sediment, and serum creatinine and blood urea nitrogen were normal.

Skin biopsy was performed which revealed the characteristic lupus band at the dermo- epidermal junction. There was mild segmental endocapillary proliferation with obliteration of the capillary lumina [Figure 3].
Figure 3: Skin biopsy showing the characteristic lupus band at the dermo-epidermal junction. Mild segmental endocapillary proliferation with obliteration of the capillary lumina is also seen.

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The anti-nuclear antibodies (ANA), anti- double-stranded DNA (anti-dsDNA) antibodies, and anti-smith (anti-sm) were strongly positive with low complement levels. Complement C3 and C4 levels were low. Renal biopsy showed class III LN with granular peripheral and mesangial deposits of IgG+++, IgA+++, and C3++ seen in all the glomeruli [Figure 4]. She was pulsed with i.v. methylprednisolone and started on oral mycophenolate mofetil (MMF) along with hydroxychloroquine. She responded well to this regimen with improvement in signs and symptoms. She was discharged with the advice to continue MMF and oral steroids. However, she was not compliant with the medications and returned to the hospital with relapse and ultimately succumbed to severe sepsis.
Figure 4: Granular peripheral and mesangial deposits of IgG+++, IgA+++, and C3++ seen in all the glomeruli.

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   Discussion Top

BSLE is an uncommon subset of SLE with an incidence of 0.26 per million population in adults.[1] BSLE occurs most commonly in females between the ages of 20 and 40 years. Less than 5% of patients with SLE develop vesiculo-bullous lesions, in isolation or, in addition to other cutaneous manifestations.[3] Clinically, BSLE is characterized by rapid onset of widespread large tense bullae to small vesicles which contain clear or hemorrhagic fluid and rupture spontaneously resulting in erosions and crusts. These blisters are distributed over the neck, face, trunk, and extremities. BSLE has been reported as an initial presentation of SLE coinciding with exacerbation of SLE and also following initiation of treatment for a SLE flare.

The diagnosis of BSLE is based on the following criteria:[4]

  1. Presence of nonscarring bullous eruptions arising over, but not limited to, sun- exposed skin
  2. Subepidermal blisters with predominantly neutrophilic infiltrate in the dermis and at the basement membrane zone
  3. Direct immunofluorescence of peri-lesional skin showing IgG, IgA, IGM, and C3 deposits at the dermo-epidermal junction
  4. Indirect immunofluorescence may be negative or positive for circulating auto- antibodies to type VII collagen.

Our patient’s skin biopsy showed typical histological and direct immunofluorescence features. This was supported by positive ANA, anti-Sm, anti-dsDNA, and low complement levels. The patient had blisters and acute glomerulonephritis at presentation. Her renal biopsy revealed class III LN. She responded well to pulse methylprednisolone and MMF along with hydroxychloroquine. This regimen was associated with improvement in signs and symptoms.

The goal in managing blistering disease is to reduce the formation of new blisters, promote healing, and prevent scarring. The treatment of cutaneous SLE includes the following:[5]

  1. Preventive (photoprotective) strategies: application of sunscreens containing titanium dioxide or zinc oxide (physical- blocking agents) and avobenzone or ecam- sule (chemical-blocking agents) of sun protection factor >30, protecting against both ultraviolet A and ultraviolet B
  2. Topical therapies with corticosteroids and calcineurin inhibitors
  3. Systemic therapies: oral amino-quinolones and antimalarials (hydroxychloroquine, chloroquine, and mepacrine) constitute the first-line drugs. Additional systemic therapies include conventional treatments such as corticosteroids, methotrexate, thalido- mide, retinoids, dapsone, azathioprine, and MMF, and newer immunomodulatory therapies such as efalizumab, anti-tumor necrosis factor agents, i.v. immunoglobulin, and rituximab
  4. Dapsone is the treatment of initial choice for BSLE. The response is usually dramatic, with cessation of new blister formation within 1–2 days and rapid healing of existing lesions.

   Conclusion Top

SLE can present with varied dermatological manifestations. Bullous lesions are a rare manifestation of SLE, which is more commonly seen in the elderly but unusual in younger age group. SLE should be entertained in the differential diagnosis of patients with bullous lesions who have other manifestations of the disease.

Conflict of interest: None declared.

   References Top

Vijayalakshmi AM, Jayavardhana A. Bullous systemic lupus erythematosus and lupus nephritis in a 10 year old boy. Indian Pediatr 2007;44:861-3.  Back to cited text no. 1
Pedro SD, Dahl MV. Direct immunofluo- rescence of bullous systemic lupus erythema- tosus. Arch Dermatol 1973;107:118-20.  Back to cited text no. 2
Kumar S, Agarwal I. Unusual presentation of childhood systemic lupus erythematosus. Pediatr Rheumatol Online J 2007;5:20.  Back to cited text no. 3
Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997;40:1725.  Back to cited text no. 4
5. Walling HW, Sontheimer RD. Cutaneous lupus erythematosus: Issues in diagnosis and treatment. Am J Clin Dermatol 2009;10:365- 81.  Back to cited text no. 5

Correspondence Address:
Parampalli Rakesh Madhyastha
Department of Nephrology, M. S. Ramaiah Memorial Hospital, Mathikere, Bengaluru - 560 054, Karnataka
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DOI: 10.4103/1319-2442.206468

PMID: 28540909

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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