Bullous Systemic Lupus Erythematosus

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Abstract

Bullous Systemic Lupus Erythematosus (BSLE) is a rare manifestation of Systemic Lupus Erythematosus (SLE), characterized histologically by subepidermal bullae with predominantly neutrophilic infiltrate and immunoglobulin (IgG, IgA, IgM) and C3 deposition at the basement membrane zone. Often associated is a heterogeneous profile of autoimmunity to components of type VII collagen. It needs to be differentiated from other subepidermal bullous diseases in view of potentially associated systemic manifestations of SLE. We report a 39-year-old lady who presented with an extensive vesicobullous eruption on face, neck, trunk, and mucosa. She was also found to have photosensitivity, significant anemia, and high antibody titers confirming SLE. Routine histopathology and immunoflorescence studies suggested BSLE and she responded favorably to dapsone. The importance of clinical and histopathological findings in confirming a diagnosis of BSLE along with the very encouraging response to dapsone therapy is highlighted.

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Bullous systemic lupus erythematosus.

Snejina Vassileva (2004)

Blistering eruptions are rare cutaneous manifestations of lupus erythematosus (LE) that may be caused by different mechanisms. Subepidermal clefting with frank vesiculation may occur in early lesions of chronic-, subacute-, and acute-cutaneous LE due to a severe vacuolar alteration of the dermoepidermal junction (DEJ), dermal edema, and lekocytoclastic vasculitis. An exaggerated example of such changes is rarely seen at the advancing edge of the annular plaques of subacute cutaneous LE with erythema-multiforme (EM)-like appearance, a condition formerly described as Rowell's syndrome. In a recently reported novel variant of LE-associated toxic epidermal necrolysis, dysregulated keratinocyte apoptosis has been proposed as an underlying mechanism. These vesiculobullous lesions are considered to be LE-specific. Blistering may also occur in LE in the context of a coexisting immunobullous disease. Pemphigus, bullous pemphigoid (BP), epidermolysis bullosa acquisita (EBA), dermatitis herpetiformis, and linear IgA bullous dermatosis have been all reported in association with LE. Their differentiation relies upon characteristic clinical, histologic, and immunopathologic features (Table 1). These blistering eruptions are rather non-specific for LE.

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Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin-6, and type VII collagen.

Cecilia L. W. Chan, D Woodley, Michael Mancini … (1999)

Bullous systemic lupus erythematosus is a generalized subepidermal blistering skin eruption in patients suffering from systemic lupus erythematosus. Type VII collagen was initially identified as the target antigen. We studied an unusual patient who had bullous systemic lupus crythematosus. The patient fulfilled the criteria of systemic lupus with an antinuclear antibody titer of 1:5120. Immunopathological testing revealed in vivo deposition of all IgG subclasses, secretory IgA1, and both light chains at the patient's skin basement membrane. The in vivo-bound IgG and IgA were localized at the hemidesmosomes and lamina densa. The patient's IgG and IgA circulating autoantibodies labeled both the epidermal roof and the dermal floor of salt-split skin and recognized the hemidesmosomal protein BP230 as well as the full-length native form and the recombinant noncollagenous domain 1 of type VII collagen (anchoring fibril). In addition, the patient's IgG autoantibodies recognized the anchoring filament proteins laminin-5 and laminin-6 (alpha3 chain and gamma2 chain). We conclude that patients with bullous systemic lupus erythematosus may have autoantibodies to multiple basement membrane components critical for epidermal-dermal junctional adhesion. Possible pathogenic mechanisms in this patient's clinical diseases include provocation of organ-specific disease (bullous disease) by systemic autoimmunity (lupus) and the "epitope spreading" immune phenomenon.

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Bullous systemic lupus erythematosus: revised criteria for diagnosis.

F Wojnarowska, J Yell, Peter J. Allen … (1995)

Blistering in systemic lupus erythematosus has been divided into three groups. A specific subgroup of 'bullous systemic lupus erythematosus' has been defined by Gammon et al. on the basis of a number of criteria. From our experience of seven patients with bullous systemic lupus erythematosus, and after reviewing the literature, we suggest that the current classification is too narrow. Our patients displayed clinical and immunohistological (based on direct and indirect immunofluorescence and Western immunoblotting) heterogeneity. Sera from two patients bound to epidermal epitopes in sodium chloride-split skin, but immunoblotting was negative. In neither of these patients could the target antigen be type VII collagen, the only antigen identified as pathogenic in this disease. Patients with epidermal binding should not be excluded from a diagnosis of bullous systemic lupus erythematosus. SLE is a disease in which there is a genetic predisposition to form antibodies to type VII collagen, along with other autoantibodies, many of which may be implicated in blistering. We suggest that the criteria for the diagnosis of BSLE should be revised. We define this disease as an acquired subepidermal blistering disease in a patient with SLE, in which immune reactants are present at the basement membrane zone on either direct or indirect immunofluorescence.

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Author and article information

Journal

Journal ID (nlm-ta): Indian J Dermatol

Journal ID (iso-abbrev): Indian J Dermatol

Journal ID (publisher-id): IJD

Title: Indian Journal of Dermatology

Publisher: Medknow Publications & Media Pvt Ltd (India )

ISSN (Print): 0019-5154

ISSN (Electronic): 1998-3611

Publication date (Print): Nov-Dec 2013

Volume: 58

Issue: 6

Page: 492

Affiliations

[1] From the Department of Dermatology, University College of Medical Sciences, Dilshad Garden, Delhi, India

[1 ] Department of Pathology, University College of Medical Sciences, Dilshad Garden, Delhi, India

Author notes

Address for correspondence: Dr. Chander Grover, Assistant Professor, 420-B, Pocket 2, Mayur Vihar, Phase-1, Delhi - 110 091, India. E-mail: chandergroverkubba@ 123456rediffmail.com

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Publisher ID: IJD-58-492d

DOI: 10.4103/0019-5154.119973

PMC ID: 3827532

SO-VID: 93011089-3767-4bd6-9143-ec0dd9982968

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This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Bullous Systemic Lupus Erythematosus

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Karger: Dermatology

Most cited references 11

Bullous systemic lupus erythematosus.

Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin-6, and type VII collagen.

Bullous systemic lupus erythematosus: revised criteria for diagnosis.

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