Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Bullous Systemic Lupus Erythematosus (BSLE) Clinical Presentation

  • Author: Hillary D Johnson-Jahangir, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Dec 15, 2015
 

History

Bullous systemic lupus erythematosus is characterized by the rapid development of a widespread vesiculobullous eruption. The blistering activity does not necessarily correlate with that of the patient's lupus erythematosus–specific skin or systemic disease, but parallel exacerbations (often involving lupus nephritis) have been described. Bullous systemic lupus erythematosus occasionally represents the initial clinical manifestation of systemic lupus erythematosus in both children and adults.[20]  

Bullous systemic lupus erythematosus may be accompanied by pruritus of variable intensity. Mucosal lesions are often painful. Patients can exhibit any of the symptoms associated with systemic lupus erythematosus. These can include the following:

  • Fever
  • Weight loss
  • Fatigue
  • Photosensitivity
  • Arthralgias
  • Arthritis
  •  Esophagitis [21]
  • Manifestations of renal, pulmonary, cardiac, and/or central nervous system disease

Patients with toxic epidermal necrolysis–like acute cutaneous lupus erythematosus often have significant systemic disease activity (eg, lupus nephritis or cerebritis).

See also Systemic Lupus Erythematosus and Neurologic Manifestations of Systemic Lupus Erythematosus.

Next

Physical Examination

Extracutaneous findings of systemic lupus erythematosus may include joint tenderness and swelling, pallor or petechiae (reflecting hematologic abnormalities), and auscultation evidence of a pulmonary or pericardial effusion.

Morphology of bullous systemic lupus erythematosus skin lesions

Blisters range from large, tense bullae (resembling bullous pemphigoid) to small, grouped vesicles (resembling dermatitis herpetiformis). They often arise on erythematous skin and may be preceded by urticarial papules and plaques. An annular or figurate configuration is occasionally observed. Bullae contain clear or hemorrhagic fluid. Rupture results in erosions and crusts, which typically heal with postinflammatory hyperpigmentation or hypopigmentation. (See the image below.)

Tense vesiculobullous lesions on the neck of a pat Tense vesiculobullous lesions on the neck of a patient with bullous systemic lupus erythematosus.

Although bullous systemic lupus erythematosus was initially defined as a nonscarring bullous disease, a mechanobullous presentation resembling classic epidermolysis bullosa acquisita has been reported. In such patients, vesicles and bullae arise within noninflamed skin of trauma-prone sites (reflecting skin fragility) and heal with milia and scarring.

Distribution of bullous systemic lupus erythematosus

Bullous systemic lupus erythematosus frequently manifests in a widespread, symmetrical distribution of skin lesions. The eruption favors the upper part of the trunk, proximal upper extremities (flexural and extensor aspects), neck, and face, but blisters can occur anywhere on the cutaneous surface. Usually, no clear association exists between sun exposure and the development of lesions, which involve sun-protected and sun-exposed sites. Blisters and erosions can also affect the oral, pharyngeal, nasal, and vulvar mucous membranes.

Lesions of classic epidermolysis bullosa acquisita–like bullous systemic lupus erythematosus are localized to trauma-prone areas, such as the dorsal hands, feet, elbows, and knees.

Lupus erythematosus–specific vesiculobullous skin disease

These vesiculobullous lesions are distinct from those of bullous systemic lupus erythematosus, representing severe variants of acute, subacute, or (rarely) discoid cutaneous lupus erythematosus. The eruptions can develop rapidly or evolve over several weeks.

In toxic epidermal necrolysis–like acute cutaneous lupus erythematosus, photodistributed diffuse or patchy erythema evolves (usually rapidly) into flaccid bullae (positive Nikolsky sign, unlike bullous systemic lupus erythematosus) and widespread, sheetlike, full-thickness epidermal detachment.

Toxic epidermal necrolysis–like subacute cutaneous lupus erythematosus manifests as widespread blistering and full-thickness epidermal detachment in the context of preexisting photodistributed annular or papulosquamous skin lesions and anti-Ro/SS-1 and/or anti-La/SS-2 antibody production. Involvement of the oral, conjunctival, and genital mucosae occurs in some cases of toxic epidermal necrolysis–like lupus erythematosus.

Erythema multiforme–like lesions occurring in the context of acute, subacute, or (as initially described by Rowell and colleagues in 1963) discoid cutaneous lupus erythematosus have been referred to as Rowell syndrome. The development of these targetoid erythematous plaques with central (or, in the setting of subacute cutaneous lupus erythematosus, peripheral) blistering and erosions is thought to represent a limited form of toxic epidermal necrolysis–like lupus erythematosus. Mucosal involvement is often observed.[22, 23, 24]

Some authors[25] identify Rowell syndrome as a rare but distinct clinical entity with specific diagnostic criteria. The diagnosis of Rowell syndrome requires the presence of all redefined major criteria and at least 1 of the minor criteria. Major criteria include the coexistence of lupus erythematous, erythema multiforme–like lesions, and a speckled pattern of antinuclear antibodies. Minor criteria include chilblains, positive anti-Ro (SS-A) or anti-La (SS-B) antibodies, and a reactive rheumatoid factor.

Vesicles or erosions at the advancing edge of active annular subacute lupus erythematosus lesions represent a relatively common observation.

Other mucocutaneous findings

Specific cutaneous lesions of acute, subacute, and chronic (discoid) cutaneous lupus are observed in a minority of patients with bullous systemic lupus erythematosus. (See also Acute Lupus Erythematosus, Subacute Cutaneous Lupus Erythematosus, and Discoid Lupus Erythematosus.)

Nonspecific cutaneous findings of lupus erythematosus, such as periungual telangiectasias, livedo reticularis, and nonscarring alopecia, also may be present. Chilblain lupus can occur in association with Rowell syndrome.

Previous
 
 
Contributor Information and Disclosures
Author

Hillary D Johnson-Jahangir, MD, PhD Clinical Assistant Professor, Department of Dermatology, University of Iowa, Roy J and Lucille A Carver College of Medicine

Hillary D Johnson-Jahangir, MD, PhD is a member of the following medical societies: American College of Mohs Surgery, American Medical Association, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Iowa Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Alice L Ye Medical Student, University of Iowa, Carver College of Medicine

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Julie V Schaffer, MD Assistant Professor of Dermatology and Pediatrics, Director of Dermatology Resident Education, Director of Pediatric Dermatology Unit, New York University School of Medicine

Disclosure: Nothing to disclose.

Ivan D Camacho, MD Dermatologist, Private Practice; Voluntary Assistant Professor of Dermatology, Department of Dermatology and Cutaneous Surgery, University of Miami, Leonard M Miller School of Medicine

Ivan D Camacho, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Florida Medical Association, International Society of Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Acknowledgements

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Michael Girardi, MD Associate Professor and Residency Director, Department of Dermatology, Yale University School of Medicine

Michael Girardi, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

References
  1. Fujimoto W, Hamada T, Yamada J, Matsuura H, Iwatsuki K. Bullous Systemic Lupus Erythematosus as an Initial Manifestation of SLE. J Dermatol. 2005 Dec. 32(12):1021-7. [Medline].

  2. Poojary S, Rais S. Bullous systemic lupus erythematosus with lupus nephritis: a rare case of a subepidermal bullous disorder in a child. Cutis. 2012 Jan. 89(1):17-21. [Medline].

  3. 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. 2004 May. 43(5):348-51. [Medline].

  4. Roustan G, Salas C, Barbadillo C, Sánchez Yus E, Mulero J, Simón A. Lupus erythematosus with an erythema multiforme-like eruption. Eur J Dermatol. 2000 Aug. 10(6):459-62. [Medline].

  5. Horne NS, Narayan AR, Young RM, Frieri M. Toxic epidermal necrolysis in systemic lupus erythematosus. Autoimmun Rev. 2006 Feb. 5(2):160-4. [Medline].

  6. Mandelcorn R, Shear NH. Lupus-associated toxic epidermal necrolysis: a novel manifestation of lupus?. J Am Acad Dermatol. 2003 Apr. 48(4):525-9. [Medline].

  7. 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].

  8. 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. 2004 Jun. 43(6):420-7. [Medline].

  9. Grabell DA, Matthews LA, Yancey KB, Chong BF. Detection of Type VII Collagen Autoantibodies Before the Onset of Bullous Systemic Lupus Erythematosus. JAMA Dermatol. 2015 May. 151 (5):539-43. [Medline]. [Full Text].

  10. 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. 2004 May. 150(5):843-51. [Medline].

  11. 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. 2007 Jun. 170(6):2009-18. [Medline]. [Full Text].

  12. 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. 2006 Jun. 126(6):1323-30. [Medline].

  13. 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. 2006 Aug. 145(2):322-31. [Medline]. [Full Text].

  14. 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. 1999 May. 135(5):569-73. [Medline].

  15. 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. 1997 Jan. 108(1):68-72. [Medline].

  16. 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. 1998 Aug. 139(2):302-6. [Medline].

  17. Hertl M, Eming R, Veldman C. T cell control in autoimmune bullous skin disorders. J Clin Invest. 2006 May. 116(5):1159-66. [Medline]. [Full Text].

  18. 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].

  19. Nasongkhla P, Pratchyapruit W, Tagami H. Bullous systemic lupus erythematosus induced by UVB: report a case. J Med Assoc Thai. 2012 Jul. 95(7):969-73. [Medline].

  20. Lourenço DM, Gomes RC, Aikawa NE, Campos LM, Romiti R, Silva CA. Childhood-onset bullous systemic lupus erythematosus. Lupus. 2014 Nov. 23 (13):1422-5. [Medline].

  21. Yogarajah M, Sivasambu B, Jaffe EA. Bullous systemic lupus erythematosus associated with esophagitis dissecans superficialis. Case Rep Rheumatol. 2015. 2015:930683. [Medline]. [Full Text].

  22. Khandpur S, Das S, Singh MK. Rowell's syndrome revisited: report of two cases from India. Int J Dermatol. 2005 Jul. 44(7):545-9. [Medline].

  23. Pandhi D, Singal A, Agarwal P. Rowell's syndrome and associated antiphospholipid syndrome. Clin Exp Dermatol. 2004 Jan. 29(1):22-4. [Medline].

  24. Shteyngarts AR, Warner MR, Camisa C. Lupus erythematosus associated with erythema multiforme: does Rowell's syndrome exist?. J Am Acad Dermatol. 1999 May. 40(5 Pt 1):773-7. [Medline].

  25. Zeitouni NC, Funaro D, Cloutier RA, Gagné E, Claveau J. Redefining Rowell's syndrome. Br J Dermatol. 2000 Feb. 142(2):343-6. [Medline].

  26. 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].

  27. 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 Jul. 27(1):79-87. [Medline].

  28. 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].

  29. Alsanafi S, Kovarik C, Mermelstein AL, Werth VP. Rituximab in the treatment of bullous systemic lupus erythematosus. J Clin Rheumatol. 2011 Apr. 17(3):142-4. [Medline].

  30. Hamminga EA, Vermeer MH. Bullous systemic lupus erythematosus responding to mycophenolate mofetil. Eur J Dermatol. 2010 Nov-Dec. 20(6):844-5. [Medline].

  31. Anyanwu CO, Ang CC, Werth VP. Oral mucosal involvement in bullous lupus. Arthritis Rheum. 2013 Oct. 65 (10):2622. [Medline].

 
Previous
Next
 
Tense vesiculobullous lesions on the neck of a patient with bullous systemic lupus erythematosus.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.