|Year : 2021 | Volume
| Issue : 3 | Page : 247-249
Bullous systemic lupus erythematosus as a presenting manifestation of systemic lupus erythematosus in a child
Sumedha Ballal1, Mary Augustine1, Rajalakshmi Tirumalae2
1 Department of Dermatology, St John's Medical College Hospital, Bengaluru, Karnataka, India
2 Department of Pathology, St John's Medical College Hospital, Bengaluru, Karnataka, India
|Date of Submission||05-Jun-2020|
|Date of Decision||02-Jul-2020|
|Date of Acceptance||21-Mar-2021|
|Date of Web Publication||30-Jun-2021|
Department of Dermatology, St John's Medical College Hospital, Sarjapur Road, Bengaluru - 560 034, Karnataka
Source of Support: None, Conflict of Interest: None
Introduction: Bullous systemic lupus erythematosus (BSLE) is a rare blistering disorder seen in the background of systemic lupus erythematosus (SLE). It can either be a presenting feature or manifest later in an established case of SLE often accompanied by lupus nephritis. Case Report: We report a case of a 12-year-old girl in whom the presenting feature of SLE was BSLE, along with hemolytic anemia. Discussion: Bullous SLE should be considered in the differential diagnosis in children presenting with vesiculobullous lesions even in the absence of established SLE
Keywords: Child; Dapsone; Blister; Anemia, hemolytic; Basement membrane
|How to cite this article:|
Ballal S, Augustine M, Tirumalae R. Bullous systemic lupus erythematosus as a presenting manifestation of systemic lupus erythematosus in a child. Indian J Paediatr Dermatol 2021;22:247-9
|How to cite this URL:|
Ballal S, Augustine M, Tirumalae R. Bullous systemic lupus erythematosus as a presenting manifestation of systemic lupus erythematosus in a child. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Jul 24];22:247-9. Available from: https://www.ijpd.in/text.asp?2021/22/3/247/319975
| Introduction|| |
Systemic lupus erythematosus (SLE) is a multisystem disease with skin manifestations that may vary from mild to life-threatening. Childhood-onset SLE (cSLE) has a prevalence of 3.3–24/100,000 children and contributes to 10%–20% cases seen in adulthood. The median age of presentation is around 11–12 years and is rarely reported in children under 5 years of age. In general, cSLE has a more severe clinical course compared to adult lupus, with higher prevalence of photosensitivity, mucocutaneous involvement, lupus nephritis, neuropsychiatric manifestations, and hematologic abnormalities. Most patients with cSLE develop cutaneous manifestations either at presentation or later, which include malar rash, mucocutaneous ulceration, and nonscarring alopecia. BSLE is an uncommon subepidermal blistering disorder that occurs in <5% of patients with SLE, characterized by an acute-onset, generalized eruption of tense vesicles and bullae; predominantly, but not restricted to, sun-exposed skin. It is rare in cSLE (<1%) with only a few cases reported in India and is often associated with lupus nephritis., We report a case of BSLE as the first manifestation of cSLE which was associated with hemolytic anemia at presentation, and no renal involvement for one year of follow-up.
| Case Report|| |
A 12-year-old girl presented with extensive bullous lesions over the body since 2 weeks, associated with moderate grade fever. There was no history suggestive of photosensitivity, oral ulcers, joint pain, or Raynaud's phenomenon. Cutaneous examination revealed extensive tense vesicles and bullae of varying sizes, arising from normal-looking skin, predominantly behind the ears, neck, chest, abdomen, proximal portion of the upper and lower extremities, periorbital area, and perioral area [Figure 1]. Some of them contained hemorrhagic fluid. Most of the lesions were irregular in shape and showed an annular configuration. Mucous membranes, hair, and nails were normal. The differential diagnoses considered were linear immunoglobulin (Ig) A disease (LAD), bullous pemphigoid, epidermolysis bullosa acquisita (EBA), and BSLE. Apart from tachypnea and tachycardia, a general and systemic examination was normal. Investigations showed hemolytic anemia (hemoglobin of 5.2g/dl, positive direct and indirect Coombs test), thrombocytopenia (12 × 103/μl), and normal prothrombin time (PT) and international normalized ratio (INR). Lupus anticoagulant and other antiphospholipid antibodies were negative. Serum creatinine was 0.5 mg/dL, and urine protein/creatinine ratio was within normal limits. Complements were decreased (C3 : 29 mg/dL and C4 : 5mg/dL). Echocardiography showed mild pericardial effusion. Anemic retinopathy without any evidence of vasculitis was seen on fundoscopy. Antinuclear antibody (ANA) was positive with a speckled pattern, ANA profile exhibiting anti-ro-52 (3+), SSA (2+), and dsDNA (1+). Skin biopsy showed subepidermal vesicles containing chiefly neutrophils, perivascular neutrophilic infiltrate, and dermal mucin deposition. There was no evidence of vasculitis. Linear deposits of Ig (IgG, IgA, and IgM) and C3 were detected at the basement membrane zone (BMZ) on direct immunofluorescence (DIF) [Figure 2] and [Figure 3]. The cutaneous findings, histopathology, and DIF were consistent with BSLE and the systemic investigations were diagnostic of SLE. She was treated with systemic steroids (Pulse methylprednisolone 10mg/kg/dose for three days) and hydroxychloroquine (150 mg). Skin lesions showed considerable improvement after methylprednisolone pulse. Azathioprine (0.5 mg/kg) and dapsone (75 mg) were added after the hemoglobin increased to 8 g/dl. At the end of 1 month, her skin lesions had completely healed, with hypopigmentation, and dapsone was stopped at 3-month follow-up. SLE activity also went into remission within 6 months of initiating therapy. The patient has been asymptomatic for the past 1 year and is on a tapering dose of oral steroids and azathioprine.
|Figure 1: Extensive, clear-fluid filled, tense vesicles and bullae arranged in an annular pattern over the neck, chest, and abdomen|
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|Figure 2: Direct immunofluorescence showing linear deposits of immunoglobulins G at the basement membrane zone (×4)|
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|Figure 3: Direct immunofluorescence showing linear deposits of immunoglobulin M at the basement membrane zone (×4)|
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| Discussion|| |
BSLE is characterized by an acute onset of generalized vesiculobullous eruption, occurring in a patient who has fulfilled the revised American Rheumatism Association (ARA) criteria or Systemic Lupus International Collaborating Clinic (SLICC) group criteria for SLE.
Lesions/blisters have a predilection for the trunk, upper extremities, supraclavicular region, face, vermillion border of the lips, and oral mucosa. Tense vesicles and bullae are seen over normal-looking and/or erythematous skin and contain clear or hemorrhagic fluid. Herpetiform vesicles, erythematous annular plaques, urticarial papules, and targetoid lesions have been described. Lesions heal with hyper- or hypopigmentation although typically without milia and scarring. Our patient had tense irregularly shaped vesicles and bullae, arising from normal-looking skin only, predominantly in an annular configuration.
DIF of BSLE demonstrates a linear or granular deposition of IgG, IgA, IgM, and C3 in the BMZ. The autoantibodies bind to the dermal side of the BMZ in sodium chloride-separated skin and are directed against the noncollagenous domain type 1 and 2 (NC1 and NC2) of type VII collagen in most patients.
Gammon and Briggaman described two immunological types of BSLE based on the presence (type 1) or absence (type 2) of circulating and/or tissue-bound antibodies against type VII collagen.
Type 1 may show circulating autoantibodies on the dermal portion of salt-split skin, with immunoblotting demonstrating antibodies against 290 kD (type VII collagen) and 145 kD (recombinant NC1 domain). Immune electron microscopy (IEM) confirms Ig deposits beneath, and on, the lamina densa. Type 2 is associated with tissue-bound antibodies in the upper dermis but not on, or just below, the lamina densa, or in association with anchoring fibrils. In addition, Yell et al. demonstrated an epidermal staining pattern in some patients with clinical and histopathological features of BSLE, suggesting a wider definition including this third variant.
Among the differential diagnoses considered, EBA was unlikely because of the absence of scaring or milia and a good response to dapsone and steroids. LAD and BSLE can appear identical on both histopathology and DIF. LAD is known to demonstrate IgG, IgM, and C3 in a minority of cases. Apart from BSLE, bullous lesions associated with specific lesions of LE show a predominant lymphocytic infiltrate along with epidermal atrophy, basal vacuolization, and thickening of BMZ.
Our case fulfilled the SLE criteria put forward by SLICC (hemolytic anemia, thrombocytopenia, positive ANA, positive Ds DNA, and low complements). In addition, criteria for BSLE suggested by Camisa and Sharma were also fulfilled. IIF, immunoblotting, and immunoelectron microscopy (IEM) were not performed due to logistic issues.
Patients with BSLE may or may not have other skin or systemic manifestations of SLE. As was in our case, majority of childhood BSLE has been reported as the initial presentation of cSLE. Most of them had associated lupus nephritis which was not seen in our patient till 1-year follow-up. Further, hemolytic anemia with childhood BSLE has not been reported to the best of our knowledge. However, hemolytic anemia may be the first manifestation of SLE, occasionally preceded by years, and is more common in cSLE. The treatment of choice in BSLE is usually dapsone. Other treatment options include prednisone, colchicine, azathioprine, antimalarials, cyclophosphamide, methotrexate, and mycophenolate mofetil. Rituximab is effective in refractory cases. Dapsone could not be started as the first option in our case because of the anemia.
To summarize, our patient presented with BSLE as an initial manifestation of SLE, along with hemolytic anemia, and responded well to high-dose steroids. Considering the frequency of BSLE being the first manifestation of cSLE, it should be considered in the differential diagnosis in children presenting with vesiclulobullous lesions, even in the absence of established SLE.
Declaration of consent
The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s) of the patient. In the form the parent(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child 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.
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[Figure 1], [Figure 2], [Figure 3]