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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 1  |  Issue : 2  |  Page : 110-112

New onset bullous lupus erythematosus in a systemic lupus erythematosus patient after initiation of hydroxychloroquin


1 Department of Medicine, Christian Medical College and Hospital, Ludhiana, Punjab, India
2 Department of Dermatology, Christian Medical College and Hospital, Ludhiana, Punjab, India
3 Department of Pathology, Christian Medical College and Hospital, Ludhiana, Punjab, India

Date of Web Publication11-Jun-2014

Correspondence Address:
Abhilasha Williams
Department of Dermatology, Christian Medical College and Hospital, Ludhiana 141 008, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2348-3334.134276

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  Abstract 

We report a case of a 14-year-old Indian girl, a diagnosed case of systemic lupus erythematosus (SLE) who developed generalized erythematous rash and joints pain for 15 days. She was prescribed hydroxychloroquin and low dose deflazacort for joint pains. Within 3 days of initiation of hydroxychloroquin she developed generalized eruptions in the form of tense, fluid-filled blisters, erosions, and crusting. Biopsy showed subepidermal blistering with a prominent neutrophilic infiltrate. Direct immunofluorescence (DIF) showed positive granular immunoglobulin (Ig)G deposition with C3 and C1q at the dermoepidermal junction which was consistent with bullous SLE (BSLE). The lesions responded dramatically to dapsone 100 mg daily.

Keywords: Bullous systemic lupus erythematosus, hydroxychloroquin, blisters


How to cite this article:
Gera C, Williams A, Thomas EA, Calton N. New onset bullous lupus erythematosus in a systemic lupus erythematosus patient after initiation of hydroxychloroquin. CHRISMED J Health Res 2014;1:110-2

How to cite this URL:
Gera C, Williams A, Thomas EA, Calton N. New onset bullous lupus erythematosus in a systemic lupus erythematosus patient after initiation of hydroxychloroquin. CHRISMED J Health Res [serial online] 2014 [cited 2019 Aug 23];1:110-2. Available from: http://www.cjhr.org/text.asp?2014/1/2/110/134276


  Introduction Top


Lupus erythematosus (LE) is a disease with a wide spectrum which ranges from benign cutaneous to life-threatening systemic involvement. The prevalence of systemic LE (SLE) is 17-48/100,000 population worldwide. [1] Skin disease occurs in up to 70% of patients during the course of the disease. [1] Classical forms of skin involvement are subacute cutaneous LE (SCLE) and chronic discoid LE (DLE). [2] Few unusual variants of cutaneous LE are bullous SLE (BSLE), linear cutaneous LE, chilblains LE, [3] LE profundus/panniculitis, LE tumidus, urticaria vasculitis, hypertrophic LE, [2] annular erythema, and papulonodular mucinosis. [4] We report a 16-year-old girl, a diagnosed case of SLE developed bullous eruptions on instituting hydroxychloroquin. Histopathology examination with direct immunofluorescence (DIF) confirmed bullous LE.


  Case Report Top


A 14-year-old girl, diagnosed case of SLE, presented with 15 days history of joints pain and erythematous generalized rash. She was diagnosed SLE because of photosensitivity, oral ulcers, malar rash, elevated antinuclear antibody (ANA), and positive double stranded deoxyribonucleic acid (dsDNA). She also had history of Raynaud's phenomenon. As she was complaining of joint pain, she was prescribed hydroxychloroquin and deflazacort 12 mg daily. On 3 rd day of starting of hydroxychloroquin, patient developed multiple blisters over arms and neck. She continued drug and her eruptions were increasing in size and number. After 3 days of onset of eruptions, patient stopped hydroxychloroquin and reported.

On physical examination, her pulse was 88/min regular, blood pressure was 110/70 mmHg, and oral temperature was 98.4°F. Oral cavity showed multiple ulcers. Skin examination revealed malar rash; diffuse faint periorbital erythema along with multiple tense vesiculobullous lesions on arms, neck, face, auricular, and upper back; and few of them filled with hemorrhagic fluid [Figure 1]. She had swelling, tenderness, and discoloration of overlying skin on small joints of hands. The examination of her head, neck, lungs, heart, and abdomen was unremarkable.
Figure 1: Multiple bullous eruptions on arm

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Investigations revealed hemoglobin was 80 g/L and white blood cell was 53 × 10 9 /L with neutrophils of 33 × 10 9 /L and lymphocytes of 19 × 10 9 /L. Her platelets were 17 × 10 9 /L. Her prothrombin time/international normalized ratio and activated partial thromboplastin time were within normal limits. Her serum creatinine was normal. ANA was positive (2.5 IU; normal ≤1.1 IU) with speckled pattern and dsDNA was elevated. Complements (C3 and C4) were low. Her direct Coombs test was positive. Urine microscopy, liver and renal function tests, enzyme-linked immunosorbent assay for human immunodeficiency virus, and ultrasonography abdomen were within normal limits.

Skin biopsy showed hyperkeratosis and subepidermal bulla formation, mononuclear cell within epidermis, melanin incontinence, and vacuolar degeneration. Edema was present in epidermis with abscesses in papillary dermis, nuclear dust around the blood vessels, and appendages [Figure 2].
Figure 2: Subepidermal bulla with neutrophilic infi ltrates (arrow) (hematoxylin and eosin, ×400)

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DIF showed immunoglobulin (IgG), C3, and C1q positivity along the dermoepidermal junction. IgM and IgA were negative. This histopathology and DIF findings were consistent with diagnosis of bullous LE.

Patient was started on dapsone 100 mg/day. Within 24 h her lesions started resolving and within 48 h all the bullae had disappeared.


  Discussion Top


BSLE is an extremely uncommon subset of LE and has specific clinical, histological, and immunopathological features. [5] It is an autoantibody-mediated blistering skin disease which occurs in patients with SLE. Molecular studies have suggested type VII collagen was the target antigen, but recently investigators have found that type VII collagen may not be the exclusive target antigen in BSLE. Researchers have demonstrated multiple other antigens, for example, laminin-5, laminin-6, and bullous pemphigoid antigen in a patient of BSLE. [6] Multiple antigenicity is described on the basis of epitope-spreading phenomenon, an immunologic event in which a primary autoimmune or inflammatory process causes tissue injury and releases previously "sequestered" antigenic epitopes, thus leading to a secondary autoimmune response to the "new" antigenic epitopes. [6]

The diagnosis of BSLE depends on histopathological examination, which shows neutrophilic dermal infiltrate and subepidermal separation. [7] DIF of perilesional skin reveals deposition of IgG with or without IgM and IgA and complement in a linear or granular band-like pattern at the basement membrane. Our patient's skin biopsy has shown typical histopathologic and DIF showed IgG, C3, and C1q deposition at dermoepidermal junction, while IgM and IgA were negative. Studies indicate that split skin is a more sensitive substrate than intact skin for detecting the antibodies and antibodies with different ultrastructural binding sites can often be differentiated from one another on split skin. [8]

In our patient eruptions developed simultaneously with hydroxychloroquin intake, although it is not a known drug to cause BSLE. Hydroxychloroquin sulfate is a synthetic antimalarial drug which is liberally used in rheumatology. Hydroxychloroquin sulfate-induced common cutaneous adverse reactions like skin pigmentation, contact dermatitis, fatal toxic epidermal necrolysis, acute generalized exanthematous pustulosis, phototoxic dermatitis, photoallergic dermatitis, and delayed hypersensitivity [9] have been reported, but not BSLE. It is possible that in our patient bullous eruptions were because of hydroxychloroquin, although we have not proved it. Other drug which possibly precipitates bullous LE is hydralzine. [10] Patients of BSLE show dramatic response to dapsone and so did our patient.

 
  References Top

1.Patel P, Werth V. Cutaneous lupus erythematosus: A review. Dermatol Clin 2002;20:373-85.  Back to cited text no. 1
    
2.Tebbe B. Clinical course and prognosis of cutaneous lupus erythematosus. Clin Dermatol 2004;22:121-4.  Back to cited text no. 2
[PUBMED]    
3.Crowson AN, Magro C. The cutaneous pathology of lupus erythematosus: A review. J Cutan Pathol 2001;28:1-23.  Back to cited text no. 3
    
4.Kuhn A, Schuppe HC, Ruzicka T, Lehmann P. Rare cutaneous manifestations of lupus erythematosus. A clinical overview. Hautarzt 2000;51:818-25.  Back to cited text no. 4
    
5.Dhir R, Desylva P, Gehi N, Malik A, Singh YD, Jagannayakulu H, et al. Pericardial effusion with vesiculobullous lesions in a young female. Bullous systemic lupus erythematosus (bullous SLE). Indian J Dermatol Venereol Leprol 2006;72:175-7.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
6.Chan LS, Lapiere JC, Chen M, Traczyk T, Mancini AJ, Paller AS, et al. Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin-6, and type vii collagen. Arch Dermatol 1999;135:569-73.  Back to cited text no. 6
    
7.Kumar S, Agarwal I. Unusual presentation of childhood Systemic Lupus Erythematosus. Pediatr Rheumatol Online J 2007;21;5:20.  Back to cited text no. 7
    
8.Gammon WR, Fine JD, Forbes M, Briggaman RA. Immunofluorescence on split skin for the detection and differentiation of basement membrane zone autoantibodies. J Am Acad Dermatol 1992;27:79-87.  Back to cited text no. 8
    
9.Pérez-Ezquerra PR, de Barrio Fernández M, de Castro Martínez FJ, Ruiz Hornillos FJ, Prieto García A. Delayed hypersensitivity to hydroxychloroquine manifested by two different types of cutaneous eruptions in the same patient. Allergol Immunopathol (Madr) 2006;34:174-5.  Back to cited text no. 9
    
10.Fleming MG, Bergfeld WF, Tomecki KJ, Tuthill RJ, Norris M, Benedetto EA, et al. Bullous systemic lupus erythematosus. Int J Dermatol 1989;28:321-6.  Back to cited text no. 10
    


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