Bullous Systemic Lupus Erythematosus (BSLE) Differential Diagnoses
- Author: Ivan D Camacho, MD; Chief Editor: Dirk M Elston, MD more...
Diagnostic Considerations
The clinical, histologic, and immunohistochemical features of bullous systemic lupus erythematosus can resemble epidermolysis bullosa acquisita (the inflammatory form more often than the classic mechanobullous form), dermatitis herpetiformis, and bullous pemphigoid.
Toxic epidermal necrolysis–like lupus erythematosus demonstrates clinical and histologic overlap with drug-induced toxic epidermal necrolysis and toxic epidermal necrolysis–like variants of acute graft versus host disease and pseudoporphyria. Usually these cases have a previous history of SLE, as well as serologic markers for SLE, and lack a history of drug ingestion.[22] A similar clinical presentation is occasionally observed in patients with fulminant linear IgA bullous dermatosis (especially the drug-induced variety).
Differential Diagnoses
- Bullous Pemphigoid
- Dermatitis Herpetiformis
- Drug-Induced Bullous Disorders
- Epidermolysis Bullosa
- Epidermolysis Bullosa Acquisita
- Erythema Multiforme
- Graft Versus Host Disease
- Hydroa Vacciniforme
- Linear IgA Dermatosis
- Porphyria Cutanea Tarda
- Pseudoporphyria
Fujimoto W, Hamada T, Yamada J, Matsuura H, Iwatsuki K. Bullous Systemic Lupus Erythematosus as an Initial Manifestation of SLE. J Dermatol. Dec 2005;32(12):1021-7. [Medline].
Daneshpazhooh M, Shahdi M, Aghaeepoor M, Hasiri G, Chams C. A comparative study of antibody titers of blister fluid and serum in patients with subepidermal immunobullous diseases. Int J Dermatol. May 2004;43(5):348-51. [Medline].
Roustan G, Salas C, Barbadillo C, Sánchez Yus E, Mulero J, Simón A. Lupus erythematosus with an erythema multiforme-like eruption. Eur J Dermatol. Aug 2000;10(6):459-62. [Medline].
Horne NS, Narayan AR, Young RM, Frieri M. Toxic epidermal necrolysis in systemic lupus erythematosus. Autoimmun Rev. Feb 2006;5(2):160-4. [Medline].
Mandelcorn R, Shear NH. Lupus-associated toxic epidermal necrolysis: a novel manifestation of lupus?. J Am Acad Dermatol. Apr 2003;48(4):525-9. [Medline].
Paradela S, Martinez-Gomez W, Fernandez-Jorge B, et al. Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus. Lupus. 2007;16(9):741-5. [Medline].
[Guideline] Finnish Medical Society Duodecim. Systemic lupus erythematosus (SLE). EBM Guidelines. Evidence-Based Medicine. Feb 2007;[Full Text].
Yang S, Gao Y, Song Y, Liu J, Yang C, Liu J, et al. The study of the participation of basement membrane zone antibodies in the formation of the lupus band in systemic lupus erythematosus. Int J Dermatol. Jun 2004;43(6):420-7. [Medline].
Ishii N, Yoshida M, Hisamatsu Y, et al. Epidermolysis bullosa acquisita sera react with distinct epitopes on the NC1 and NC2 domains of type VII collagen: study using immunoblotting of domain-specific recombinant proteins and postembedding immunoelectron microscopy. Br J Dermatol. May 2004;150(5):843-51. [Medline].
Chen M, Doostan A, Bandyopadhyay P, et al. The cartilage matrix protein subdomain of type VII collagen is pathogenic for epidermolysis bullosa acquisita. Am J Pathol. Jun 2007;170(6):2009-18. [Medline]. [Full Text].
Woodley DT, Ram R, Doostan A, et al. Induction of epidermolysis bullosa acquisita in mice by passive transfer of autoantibodies from patients. J Invest Dermatol. Jun 2006;126(6):1323-30. [Medline].
Chen L, Peterson JD, Zheng WY, Lin SX, Chan LS. Autoimmunity to type VII collagen in SKH1 mice is independent of regulatory T cells. Clin Exp Immunol. Aug 2006;145(2):322-31. [Medline]. [Full Text].
Chan LS, Lapiere JC, Chen M, 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. May 1999;135(5):569-73. [Medline].
Chen M, Chan LS, Cai X, et al. Development of an ELISA for rapid detection of anti-type VII collagen autoantibodies in epidermolysis bullosa acquisita. J Invest Dermatol. Jan 1997;108(1):68-72. [Medline].
Fujii K, Fujimoto W, Ueda M, Makino E, Arata J. Detection of anti-type VII collagen antibody in Sjögren's syndrome/lupus erythematosus overlap syndrome with transient bullous systemic lupus erythematosus. Br J Dermatol. Aug 1998;139(2):302-6. [Medline].
Hertl M, Eming R, Veldman C. T cell control in autoimmune bullous skin disorders. J Clin Invest. May 2006;116(5):1159-66. [Medline]. [Full Text].
Ting W, Stone MS, Racila D, Scofield RH, Sontheimer RD. Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus and the spectrum of the acute syndrome of apoptotic pan-epidermolysis (ASAP): a case report, concept review and proposal for new classification of lupus erythematosus vesiculobullous skin lesions. Lupus. 2004;13(12):941-50. [Medline].
Khandpur S, Das S, Singh MK. Rowell's syndrome revisited: report of two cases from India. Int J Dermatol. Jul 2005;44(7):545-9. [Medline].
Pandhi D, Singal A, Agarwal P. Rowell's syndrome and associated antiphospholipid syndrome. Clin Exp Dermatol. Jan 2004;29(1):22-4. [Medline].
Shteyngarts AR, Warner MR, Camisa C. Lupus erythematosus associated with erythema multiforme: does Rowell's syndrome exist?. J Am Acad Dermatol. May 1999;40(5 Pt 1):773-7. [Medline].
Zeitouni NC, Funaro D, Cloutier RA, Gagné E, Claveau J. Redefining Rowell's syndrome. Br J Dermatol. Feb 2000;142(2):343-6. [Medline].
Paradela S, Martínez-Gómez W, Fernández-Jorge B, et al. Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus. Lupus. 2007;16(9):741-5. [Medline].
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. Jul 1992;27(1):79-87. [Medline].
Malcangi G, Brandozzi G, Giangiacomi M, Zampetti M, Danieli MG. Bullous SLE: response to methotrexate and relationship with disease activity. Lupus. 2003;12(1):63-6. [Medline].
Alsanafi S, Kovarik C, Mermelstein AL, Werth VP. Rituximab in the treatment of bullous systemic lupus erythematosus. J Clin Rheumatol. Apr 2011;17(3):142-4. [Medline].
Hamminga EA, Vermeer MH. Bullous systemic lupus erythematosus responding to mycophenolate mofetil. Eur J Dermatol. Nov-Dec 2010;20(6):844-5. [Medline].

