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CASE REPORT
Year : 2016  |  Volume : 17  |  Issue : 2  |  Page : 139-141

Bullous systemic lupus erythematosus: An uncommon diagnosis in an 8-year-old girl


Department of Dermatology, Venereology and Leprosy, School of Tropical Medicine, Kolkata, West Bengal, India

Date of Web Publication30-Mar-2016

Correspondence Address:
Dipayan Sengupta
Department of Dermatology, Venereology and Leprosy, School of Tropical Medicine, 108 Chittaranjan Avenue, Kolkata - 700 073, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.172468

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  Abstract 

Bullous systemic lupus erythematosus (BSLE) is a rare manifestation of SLE, characterized clinically by tense bullae involving photo-exposed areas and histologically by subepidermal bullae with predominantly neutrophilic infiltrate and immunoglobulin (IgG, IgA, and IgM) and C3 deposition at the dermo-epidermal junction indirect immunoflurosence. Specific diagnostic criteria for this subtype have been developed. However, the presentation in children is relatively uncommon. We report an 8-year-old girl diagnosed by histopathology and immunological examination as a case of BSLE without any clinical feature of SLE except photosensitivity. This highlights the importance of considering BSLE as a differential diagnosis in case of children presenting with tense bullae.

Keywords: Bullae in childhood, bullous systemic lupus erythematosus, lupus nephritis


How to cite this article:
Lenka U, Sengupta D, Sardar S, Ghosh S. Bullous systemic lupus erythematosus: An uncommon diagnosis in an 8-year-old girl. Indian J Paediatr Dermatol 2016;17:139-41

How to cite this URL:
Lenka U, Sengupta D, Sardar S, Ghosh S. Bullous systemic lupus erythematosus: An uncommon diagnosis in an 8-year-old girl. Indian J Paediatr Dermatol [serial online] 2016 [cited 2019 Nov 17];17:139-41. Available from: http://www.ijpd.in/text.asp?2016/17/2/139/172468


  Introduction Top


Bullous systemic lupus erythematosus (BSLE) is a rare and distinct subtype of SLE, which occurs mostly in the third decade. It presents with specific clinical, histopathological, and immunological criteria. In children, however, it is very rare making it an uncommon differential diagnosis in case of a child presenting with tense bullae.


  Case Report Top


An 8-year-old girl presented with multiple discrete clear fluid filled tense eruptions, erosions and healed dark patches over face, neck, abdomen, and extremities of 6 months duration. It started as perioral erosion and gradually involved other regions. The extremities were last to be involved. The eruptions were not limited to photo-exposed areas only. There is also associated complain of itching and photophobia but no complaint of fever or joint pain or history suggestive of Raynaud's phenomenon, or butterfly rash was revealed. Birth history and family history were nonsignificant. The patient was not on any chronic medication. No history of seizure, psychosis or history of trauma at the site of blisters was present.

On examination, she had multiple tense bulla, erosion and postinflammatory patches, involving the face, neck, abdomen, and extremities [Figure 1] and [Figure 2]. Nikolsky's sign was negative. Multiple postinflammatory scaly patches were seen over trunk and extremities. Scarring was absent. Hair and nail examination was within normal limit. No malar rash was seen. Cardiovascular, neurological, and respiratory system examination were within normal limits. Abdominal examination was also normal.
Figure 1: Multiple bullous lesions over perioral region

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Figure 2: Multiple blisters and postinflammatory hypopigmented patches involving face and anterior trunk

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Investigations revealed normal complete blood count except for hemoglobin which was 9.8 g/dl. Erythrocyte sedimentation rate was 74 mm at 1 h and C-reactive protein was negative. Serum electrolytes and blood sugar was normal. Antinuclear antibody was positive in 1:100 dilution with coarse speckled pattern and anti-ds-DNA was 138 IU/ml (more than 75 IU/ml is considered positive). Among other antibody tests anti-ro-52 (2+), antiribosomal-p-protein (3+) and antihistone antibody (3+) was positive. Among blood immunoglobulins E (IgE) was mildly elevated (335 IU/ml). Urine routine and 24 h protein examination showed proteinuria (3+) and presence of granular cast. Her international normalized ratio and prothrombin time were within normal limits. However, the renal biopsy could not be performed because of the denial from the patient's family.

Skin biopsy from these bullous lesions revealed subepidermal cleft with a few neutrophil and eosinophils [Figure 3] and [Figure 4]. The infiltrates extends along eccrine coils. Epidermal roof showed confluent necrosis in some areas. No mucin or viral inclusion body is seen. Direct immunofluorescence showed granular IgA and IgG deposition along dermo-epidermal junction with IgG > IgA. Also, perivascular IgG deposition was seen in the dermis. She was diagnosed to have BSLE. Indirect immunofluorescence was not done.
Figure 3: Photomicrograph (×4) showing subepidermal separation with inflammatory cells in papillary dermis and epidermal necrosis

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Figure 4: Photomicrograph (×10) showing part of subepidermal separation with predominantly neutrophilic and eosinophilic along with few lymphocytes in the upper dermis

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She was treated with prednisolone 2 mg/kg/day and dapsone 50 mg for her skin lesions. Significant improvement was seen within 2 weeks.


  Discussions Top


BSLE is a rare subepidermal blistering disease that occurs in a subset of patients with SLE.[1],[2] Cutaneous manifestations, though common in SLE patients BSLE is very rare (occurred <1% of patients with SLE).[3],[4],[5]

BSLE patients present with a rapid, widespread development of tense fluid-filled bullous lesions apart from other features of SLE. Histologically, a subepidermal blister, with a predominantly neutrophilic dermal infiltrate and only occasional eosinophils, characterizes BSLE. Immunofluorescence examination may show deposition of IgG, IgA, C3, and C1q along dermo-epidermal junction.[5],[6] Apart from this typical clinical and histological presentation of BSLE, Camisa and Sharma proposed the diagnostic criteria for BSLE. It includes a diagnosis of SLE based on the following criteria of the American College of Rheumatology (ACR); vesicles and bullae mainly located on sun exposed areas; the histopathology is characterized by subepidermal bullae with microabscesses of neutrophils in the dermal papillae, similar to those found in dermatitis herpetiformis; and deposition of IgG, IgM or both, and often IgA in the basement membrane zone.[7]

However, according to Gammon and Briggaman,[8] the minimum criteria for the diagnosis of BSLE are: (1) Meet the criteria for SLE of the American Rheumatism Association, (2) presence of nonscarring acquired bullous eruption on sun exposed areas, but not limited to these sites, (3) evidence of subepidermal blister with neutrophilic infiltrate in histology, (4) deposition of IgG, IgM, IgA, and C3 at the basement membrane zone, (5) circulating autoantibodies against Type VII collagen confirmed by salt-split skin indirect immunofluorescence or immunoprecipitation, and (6) presence of Ig deposits with anchoring fibrils/Type VII collagen by immunoelectron microscopy.

Although BSLE may exhibit any of the symptoms associated with SLE, the onset and course of blistering eruption do not necessarily parallel the activity of the systemic involvement.[8]

Now in our case, the child is fulfilling the SLE criteria put forward by ACR (photosensitivity, antinuclear antibody, anti-ds-DNA antibody, 3+ proteinuria and granular cast in urine), and most other criteria suggested by both of that of Camisa and Sharma, as well as Gammon and Briggaman (nonscarring bullous starting from photo-exposed areas that is, face, subepidermal blister with neutrophilic infiltrate, IgG and IgA deposition along dermoepidermal junction). Inflammatory epidermolysis bullosa acquisitica was ruled out as the deposition was granular in nature. The renal involvement points toward lupus nephritis but could not be documented because the patient was not willing to undergo renal biopsy. Also, the circulating autoantibodies against collagen VII could not be measured due to the limitation of logistics.

BSLE is children is a very rare entity, only very few cases have been reported from India so far.[9],[10],[11],[12],[13] However, one reason may be difficulty in establishing the diagnosis due to limited laboratory facilities and other infrastructures in most part of India. We are reporting this case for its relatively rare occurrence in reported literature. Considering the systemic nature of SLE, BSLE should always be considered as a differential in case of children presenting with tense bullae even if it is not started from photo-exposed areas.

Declaration of Patient Consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/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

Nil.

Conflicts of Interest

There are no conflicts of interest.

 
  References Top

1.
Yung A, Oakley A. Bullous systemic lupus erythematosus. Australas J Dermatol 2000;41:234-7.  Back to cited text no. 1
    
2.
Rothfield N, Sontheimer RD, Bernstein M. Lupus erythematosus: systemic and cutaneous manifestations. Clin Dermatol 2006;24:348-62.  Back to cited text no. 2
    
3.
Tebbe B. Clinical course and prognosis of cutaneous lupus erythematosus. Clin Dermatol 2004;22:121-4.  Back to cited text no. 3
    
4.
Hall RP, Lawley TJ, Smith HR, Katz SI. Bullous eruption of systemic lupus erythematosus. Dramatic response to dapsone therapy. Ann Intern Med 1982;97:165-70.  Back to cited text no. 4
[PUBMED]    
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
    
6.
Vassileva S. Bullous systemic lupus erythematosus. Clin Dermatol 2004;22:129-38.  Back to cited text no. 6
    
7.
Camisa C, Sharma HM. Vesiculobullous systemic lupus erythematosus. Report of two cases and a review of the literature. J Am Acad Dermatol 1983;9:924-33.  Back to cited text no. 7
    
8.
Gammon WR, Briggaman RA. Bullous SLE: a phenotypically distinctive but immunologically heterogeneous bullous disorder. J Invest Dermatol 1993;100:28S-34S.  Back to cited text no. 8
    
9.
Grover C, Khurana A, Sharma S, Singal A. Bullous systemic lupus erythematosus. Indian J Dermatol 2013;58:492.  Back to cited text no. 9
[PUBMED]  Medknow Journal  
10.
Kumar S, Agarwal I. Unusual presentation of childhood systemic lupus erythematosus. Pediatr Rheumatol Online J 2007;5:20.  Back to cited text no. 10
    
11.
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. 11
    
12.
Poojary S, Rais S. Bullous systemic lupus erythematosus with lupus nephritis: a rare case of a subepidermal bullous disorder in a child. Cutis 2012;89:17-21.  Back to cited text no. 12
    
13.
Sirka CS, Padhi T, Mohanty P, Patel DK, Parida PR, Kar CR. Bullous systemic lupus erythematosus: response to dapsone in two patients. Indian J Dermatol Venereol Leprol 2005;71:54-6.  Back to cited text no. 13
[PUBMED]  Medknow Journal  


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



 

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