Histologically, BSLE is characterized by separation at the BMZ, with IgG, IgM, or IgA deposition on the dermal side of BMZ-split skin, and a neutrophilic infiltrate in the superficial dermis. All material on this website is protected by copyright, Copyright 1994-2023 by WebMD LLC. Clin Exp Immunol. Grabell DA, Matthews LA, Yancey KB, Chong BF. 1998-2023 Mayo Foundation for Medical Education and Research (MFMER). J Am Acad Dermatol 2013;68(3):e8995. [QxMD MEDLINE Link]. [ 1, 2, 3, 4]. 150(5):843-51. Lancet 2017;389(10083):203140. Bullous pemphigoid is a disease that causes blisters, which can develop anywhere on the skin. Alice L Ye Medical Student, University of Iowa, Carver College of MedicineDisclosure: Nothing to disclose. The authors made the following disclosures: VPW has consulted for Roche/Genentech and had research grants from Roche/Genentech. Highlight selected keywords in the article text. Pemphigus causes blisters on your skin and mucous membranes. 18. Mandelcorn R, Shear NH. Overview What is a lupus rash? 2009 May;2(5):19-28. 2017 Apr 22;389(10079):1586-8. Sarah Sweeney Pinney, MD, FAAD is a member of the following medical societies: American Academy of Dermatology, Texas Dermatological Society, Texas Medical Association, Women's Dermatologic SocietyDisclosure: Nothing to disclose. The exact immunologic pathways involved in the production of the autoantibodies that cause BSLE have not been completely elucidated. Diagnostic criteria for bullous SLE requires: The differential diagnosis of bullous SLE is SLE with blisters. [QxMD MEDLINE Link]. https://www.merckmanuals.com/professional/dermatologic-disorders/bullous-diseases/bullous-pemphigoid. The blistering activity does not necessarily correlate with that of the. 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. GS and MA contributed equally to the writing of this article. 2015. Make your tax-deductible gift and be a part of the cutting-edge research and care that's changing medicine. Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune blistering disorder that typically manifests as an acute vesiculobullous eruption in a patient with known systemic lupus erythematosus (SLE). Rituximab is an immunoglobulin G1 (IgG1) kappa monoclonal antibody composed of a murine (mouse) variable region (Fab portion) that is fused onto a human constant region (Fc portion). 2017 May. Targeted anti-complement therapy may prove to be a complementary treatment for bullous pemphigoid. Dermatol World Insights & Inquires. modify the keyword list to augment your search. [QxMD MEDLINE Link]. Lupus-associated toxic epidermal necrolysis: a novel manifestation of lupus?. 2014;71(1):92-9. Pathologic events that may lead to the . Dermatology. This type usually begins with blisters in your mouth and then on your skin or genital mucous membranes. Br J Dermatol 1995; 132: 9218. Gual A, Guilabert A, Iranzo P, et al. Roustan G, Salas C, Barbadillo C, Snchez Yus E, Mulero J, Simn A. Lupus erythematosus with an erythema multiforme-like eruption. Bullous systemic lupus erythematosus frequently manifests in a widespread, symmetrical distribution of skin lesions. Culton DA, Zhi L, Diaz LA. Dyshidrosiform bullous pemphigoid. Medicina (Kaunas). . The rash often itches. Some authors 2004. 1998 Aug. 139(2):302-6. Frampton JE. Mayo Clinic on Incontinence - Mayo Clinic Press, Mayo Clinic on Hearing and Balance - Mayo Clinic Press, FREE Mayo Clinic Diet Assessment - Mayo Clinic Press, Mayo Clinic Health Letter - FREE book - Mayo Clinic Press, Book: Mayo Clinic Family Health Book, 5th Edition, Newsletter: Mayo Clinic Health Letter Digital Edition, Itching skin, weeks or months before blisters form, Large blisters that don't easily rupture when touched, often along creases or folds in the skin, Skin around the blisters that is normal, reddish or darker than normal, Small blisters or sores in the mouth or other mucous membranes (benign mucous membrane pemphigoid). The rash can appear in one or two places on your skin or be widespread. Systemic lupus erythematosus (SLE) is a heterogeneous disease associated with multiple acute or chronic cutaneous manifestations, including the relatively rare category of bullous lupus. Bullous systemic lupus erythematosus (BSLE) is a rare blistering presentation of systemic lupus erythematosus, typically affecting women with the highest incidence in those of African descent. J Med Assoc Thai. Heres how long botox tends to last. [QxMD MEDLINE Link]. Search for Similar Articles Rowell's syndrome revisited: report of two cases from India. Patients successfully treated with methotrexate or rituximab have been reported [811]. Proposed therapeutic ladder for BSLE. The deep periadnexal inflammation is lymphocytic, with scattered neutrophils. If you want to diminish a noticeable scar, know these 10 things before having laser treatment. 2017 Dec. 15 (12):1211-1226. Subacute cutaneous lupus erythematosus (SCLE) is a subtype of cutaneous lupus erythematosus. VPW participated in the writing of the article and critically revised it. Systemic lupus is a type of autoimmune disease where your immune system attacks your body, including your internal organs. Blistering skin disorders are characterized by the presence of fluid-filled lesions on the skin that occur as a result of a loss of adhesion between cells within the epidermis ( acantholysis ), edema between epidermal cells ( spongiosis ), or disassociation of the epidermis and dermis. There are several clinical types: Cutaneous lichen planus Mucosal lichen planus Lichen planopilaris Lichen planus of the nails Lichen planus pigmentosus Lichenoid drug eruption. J Dtsch Dermatol Ges. Bullous pemphigoid is a disease that causes blisters, which can develop anywhere on the skin. Their appearance can range from small, grouped vesicles reminiscent of lesions in dermatitis herpetiformis to large, tense blisters, similar to bullous pemphigoid. Involvement of the oral, conjunctival, and genital mucosae occurs in some cases of toxic epidermal necrolysislike lupus erythematosus. Malcangi G, Brandozzi G, Giangiacomi M, et al. Malcangi G, Brandozzi G, Giangiacomi M, Zampetti M, Danieli MG. Bullous SLE: response to methotrexate and relationship with disease activity. [QxMD MEDLINE Link]. References Bullous lupus; bullous systemic lupus erythematosus; type VII collagen; vesiculobullous skin disease. About 10% of all lupus cases are cutaneous and 65% of people with systemic lupus will develop skin lupus. Am J Pathol. 18:317-319. The rash often itches. Pons-Estel GJ, Quintana R, Alarcn GS, et al. Use these professionally produced online infographics, posters, and videos to help others find and prevent skin cancer. [QxMD MEDLINE Link]. Some cases may be triggered by: Bullous pemphigoid most commonly occurs in older adults, and the risk increases with age. The development of vesiculo-bullous lesions may be associated with a high morbidity, hence they warrant an urgent investigation, including a skin biopsy to identify the diagnosis and initiate prompt treatment . Bullous pemphigoid secondary to pembrolizumab mimicking toxic epidermal necrolysis. JAAD Case Reports 2020;6:400-2. Br J Dermatol. The incidence of bullous SLE was estimated to be 0.22 and 0.26 cases per million per year in France and Singapore. If you develop blisters, the blisters tend to come and go. Patients with BSLE have concomitant internal organ involvement in 6990% of reported cases.11 Typically, extracutaneous findings are simultaneously present at the time of the diagnosis of BSLE, but additional findings may develop after the disease has been identified. Eur J Dermatol. It can smooth out deep wrinkles and lines, but the results arent permanent. Cohen PR. Subacute cutaneous lupus: Ring-shaped or scaly rashes appear, usually on your back, chest or neck. Dermatology Made Easybook. Ann Intern Med 1982;97(2):165170. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2NTQwMi1jbGluaWNhbA==. You can expect permanent results in all but one area. DermNet provides Google Translate, a free machine translation service. [QxMD MEDLINE Link]. The bullae can arise from erythematous macules, inflammatory plaques, or previously normal skin. 2017 Nov 4. Fitzpatricks Dermatology. Serious adverse events are rare, the most common being bacterial sepsis. Pediatr Dermatol. This mostly includes lupus nephritis (classes IIIV, lifetime prevalence of up to 90%), arthralgias/arthritis, and cytopenias, while serositis and neuropsychiatric involvement are rare. The sun, heat, and humidity can all trigger rosacea and lead to flare-ups. 2016 Oct. 28 (5):660-662. 2003 Apr. Epidemiology and pathogenesis of bullous pemphigoid and mucous membrane pemphigoid. These dermatologists' tips tell you how to protect your skin. 4). 2004 Jan. 29(1):22-4. 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 2017 Jan. 34 (1):e54-e56. You could have new blisters every day. Dirk M Elston, MD is a member of the following medical societies: American Academy of DermatologyDisclosure: Nothing to disclose. 126(6):1323-30. Bullous systemic lupus erythematosus (SLE) is an autoimmune subepidermal blistering disease that occurs in patients with SLE. Mucosal lesions are often painful. 2012 Jul. Other laboratory findings that may be seen in BSLE due to underlying SLE include anemia, leukopenia, thrombocytopenia, low levels of complement (eg, C3, C4, CH50), positive protein or cellular casts in urine, and increased erythrocyte sedimentation rate. However, the choice of dosing regimen is often dictated by insurance (Fig. Serration pattern analysis of DIF microscopy differentiates tissue bound auto-antibodies against type VII collagen, where a u-serrated pattern is seen, from all other anti-DEJ antibodies where an n-serrated pattern is seen [7]. Treatment usually helps heal the blisters and ease any itching. J Am Acad Dermatol. Incidence of bullous pemphigoid and mortality of patients with bullous pemphigoid in Olmsted County, Minnesota, 1960 through 2009. J Am Acad Dermatol. Br J Dermatol 2004; 151: 11218. 2021 Jun. Blisters range from large, tense bullae (resembling bullous pemphigoid) to small, grouped vesicles (resembling dermatitis herpetiformis). 2006 Feb. 5(2):160-4. Newer treatments for non-Hodgkins lymphoma: monoclonal antibodies. Yogarajah M, Sivasambu B, Jaffe EA. Malcangi G, Brandozzi G, Giangiacomi M, Zampetti M, Danieli MG. Bullous SLE: response to methotrexate and relationship with disease activity. DEFINITION. Maggio MC, Corsello G, Prinzi E, Cimaz R. Systemic Lupus Erythematosus and Bullous Pemphigoid with Dramatic Response to Dapsone. ; GLADEL. October 2019. Do you know which one? Am J Clin Dermatol 2020;21:14956. Where dapsone fails, prednisolone (systemic steroids) and azathioprine are used. Repeated use of rituximab over a 5-year period was safe and effective for BSLE flares in an 18-year-old African American woman with childhood onset of BSLE extending into her late teens and early adulthood.36 In this case, rituximab given via the RA dosing regimen was the only agent to consistently result in the resolution of both cutaneous and systemic disease during multiple episodes of BSLE.36 Presence of active underlying SLE should be considered in the management of individuals with BSLE (Fig. If steroids (eg, prednisone) are to be used, it would be prudent to combine them with steroid-sparing agents, as is common with other immunobullous diseases (Fig. Clinical, histological, immunological presentations and outcomes of. Br J Dermatol. Pediatr Dermatol. Other times, the disease calms down, so you may not see blisters for a while. In toxic epidermal necrolysislike 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. to maintaining your privacy and will not share your personal information without Report of two cases and a review of the literature. The complications of bullous SLE are those of the underlying SLE. 12(1):63-6. Hermann Pinkus Memorial Lecture1992. Lancet Haematol 2021;8(8):e593604. [QxMD MEDLINE Link]. The cartilage matrix protein subdomain of type VII collagen is pathogenic for epidermolysis bullosa acquisita. Bullous pemphigoid (BUL-us PEM-fih-goid) is a rare skin condition that causes large, fluid-filled blisters. Camisa C, Sharma HM. Bernard P, Vaillant L, Labeille B, et al. Its also possible to develop blisters and never see a rash. Alsanafi S, Kovarik C, Mermelstein AL, et al. BP 230, BP 180, or laminin 5 or 6 are detected in type II bullous SLE. Blisters can develop on other areas like inside the mouth, but this is rare. Detection of anti-type VII collagen antibody in Sjgren's syndrome/lupus erythematosus overlap syndrome with transient bullous systemic lupus erythematosus. 5. Lupus is an autoimmune condition that causes pain and swelling throughout your body. The rash can appear in one or two places on your skin or be widespread. It can smooth out deep wrinkles and lines, but the results arent permanent. Other autoantibodies that may be detected include anti-double stranded DNA, anti-Smith, anti-Sjgrens-syndrome-related antigen A, anti-Sjgrens-syndrome-related antigen B, and anticardiolipin. BMZ, basement membrane zone; BP, bullous pemphigoid; BSLE. arrow-right-small-blue Hall RP, Lawley TJ, Smith HR, Katz SI. References [Full Text]. your express consent. Rituximab: applications in dermatology. JAK inhibitors are helping patients with alopecia areata, eczema/atopic dermatitis, psoriasis, and vitiligo. Lupus 2004;13(12):94150. Toxic epidermal necrolysislike 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. Bernard P, Borradori L. Pemphigoid group. In: Bolognia JL, et al. SLE affects the skin and other internal organs. Blisters may occur anywhere, but often develop on flexural areas of your skin, such as under your armpits (axilla), around your groin or on your stomach . Figure 1 was provided by Dr Julia Rood, MD, PhD. Although bullous systemic lupus erythematosus was initially defined as a nonscarring bullous disease, a mechanobullous presentation resembling classic epidermolysis bullosa acquisita has been reported. Blistering can be due to various medications, prescribed or over-the-counter, natural or synthetic. Along with itchy skin, some people develop a rash that can look like hives (or large welts), as shown here. 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 MedicineDisclosure: Nothing to disclose. In bullous SLE, tense vesicles, bullae and erosions arise on normal or erythematous skin, usually in sun-exposed sites. As with other immunobullous diseases, the use of anti-CD20 monoclonal antibodies has been safe and effective.25 Initiation/augmentation of immunosuppressants (with or without dapsone therapy) due to systemic manifestations of SLE may result in the concurrent improvement of bullous lesions in BSLE.26 Most patients respond to systemic glucocorticoids. On a molecular level, most variants of CLE are characterized by a lichenoid tissue reaction as a result of keratinocyte, endothelial cell, and dendritic cell activation [ 1 ]. BSLE is a rare manifestation of SLE that typically presents in patients with an existing SLE diagnosis, although reports of BSLE in association with new-onset SLE have been reported.5 BSLE results from autoantibodies to type VII collagen located at the BMZ.2 Type VII collagen plays a key role in connecting the dermis to the epidermis by acting as an anchoring fibril and cross-linking the lamina densa and dermal matrix.2 Thus, the autoantibodies against type VII collagen weaken the basement membrane-dermal adhesion, leading to the appearance of subepidermal blisters.6 These autoantibodies specifically target the type VII collagen noncollagenous domains types 1 and 2 (NC1 and NC2) found in the BMZ.2 Other autoantibodies that may play a role in the development of BSLE include bullous pemphigoid (BP) 180, BP 230, laminin 5, and laminin 6.7 It has been postulated that epitope spreading accounts for the antibody targets that exist in BSLE beyond type VII collagen.2 The main autoimmune insult against type VII collagen could expose otherwise hidden antigens, leading to the increased number of autoantibodies found to be associated with BSLE.2, It is likely that immunoglobulin deposition causes the lamina densa portion of the basement membrane to separate from the upper dermis, resulting in bullae formation.2 Type VII collagen autoantibodies may block or weaken the anchoring fibril connection to the lamina densa and anchoring plaques by interfering with extracellular matrix ligands, causing ineffective adhesion of the dermis to the lamina densa.2 Antibodies present near the NC2 region may interfere with the antiparallel dimer alignment of type VII collagen, disrupting adherence to the dermis.2 In vitro studies have also shown that type VII collagen autoantibodies can activate complement leading to the formation of complement-dependent peptides that induce neutrophil-dependent proteolysis at the BMZ.2, The clinical presentation of BSLE consists of an acute onset generalized vesiculobullous eruption in patients who meet criteria for SLE (Fig.
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