Autopsy and Case Reports
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Article / Clinical Case Report

Bullous systemic lupus erythematosus – a case report

Eduardo Messias Hirano Padrão; Lucas Faria Teixeira; Celina Wakisaka Maruta; Valéria Aoki; Aloisio Souza Felipe da Silva; Elizabeth In Myung Kim; Luciana Avena Smelli

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Skin involvement in systemic lupus erythematosus (SLE) occurs in more than 75% of patients with this condition. Vesicles and blisters in lupus erythematosus (LE) may be present in SLE secondary to interface vacuolar changes in the epidermis, in discoid LE also secondary to vacuolar epidermal changes, and in bullous LE secondary to antibodies anti-collagen VII deposits with neutrophilic aggregates. In addition, blisters can occur due to the association of SLE with other autoimmune blistering diseases (e.g. bullous pemphigoid). BSLE is a rare blistering disease that mainly occurs in females (30–40 years old), and less frequently in children and adolescents. The most common presentation is rapid and widespread development of tense vesicles and bullae over erythematous macules or plaques. Preferential sites are: superior trunk, proximal superior limbs, and face (lips) with symmetrical distribution. Mucosal involvement is common on perioral, pharyngeal, laryngeal, and genital areas. The involvement of sun-exposed areas is not mandatory. The lesions usually progress with no scarring, but hypo or hyperchromia may be present. We report an 18-year-old female patient with blistering lesions at admission, who was diagnosed with BSLE. She was initially treated with systemic prednisone and hydroxychloroquine. Her condition evolved with relapsing lesions, which required the introduction of Dapsone. The authors emphasize the relevance of recognizing BSLE—a rare presentation of SLE—which may evolve with marked clinical presentation.


Lupus Erythematosus, Systemic, Blister, Dapsone, Diagnosis, Therapeutics


Watanabe T, Tsuchida T. Classification of lupus erythematosus based upon cutaneous manifestations. Dermatological, systemic and laboratory findings in 191 patients. Dermatology. 1995;190(4):277-83. []. [PMID:7655105]

Lourenço DM, Gomes RC, Aikawa NE, Campos LM, Romiti R, Silva CA. Childhood-onset bullous systemic lupus erythematosus. Lupus. 2014;23(13):1422-5. []. [PMID:25074872]

Nico MMS, Lourenço SV. Multiple Blisters Along the Lip Vermilion are a Clue to Bullous Lupus Erythematosus. Acta Derm Venereol. 2012;92(4):404-5. []. [PMID:22293956]

Bernard P, Vaillant L, Labeille B, et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Arch Dermatol. 1995;131(1):48-52. []. [PMID:7826096]

Li AW, Weed JG, Stamey CR, Subtil A, Tomayko MM, Antaya RJ. Bullous systemic lupus erythematosus in a 6‐year‐old boy. Pediatr Dermatol. 2017;34(6):e313-6. []. [PMID:28851077]

Maggio MC, Corsello G, Prinzi E, Cimaz R. Systemic lupus erythematosus and bullous pemphigoid with dramatic response to dapsone. Am J Case Rep. 2017;18:317-9. []. [PMID:28352068]

Mahesh E, Madhyastha PR, Varma V, Gurudev KC, Gireesh MS, Bande SR. Unusual presentation of systemic lupus erythematosus. Saudi J Kidney Dis Transpl. 2017;28(3):653-6. []. [PMID:28540909]

Juhász M, Rogge M, Chen M, Czernik A, Kim SJ, Geller L. Case of pediatric bullous systemic lupus erythematosus treated with intravenous immunoglobulin. Pediatr Dermatol. 2017;34(1):e54-6. []. [PMID:27778386]

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;151(5):539-43. []. [PMID:25671758]

Christodoulou G, Powell M, Nguyen V, Watters K, Jafarian F. An atypical case of bullous systemic lupus erythematosus in a 16‐year‐old boy. Pediatr Dermatol. 2014;31(6):e164-6. []. [PMID:25040539]

Liu KL, Shen J, Yang C, Chen YJ. Bullous systemic lupus erythematosus in a child responding to Dapsone. Pediatr Dermatol. 2014;31(4):e104-6. []. [PMID:24846529]

Burke KR, Green BP, Meyerle J. Bullous lupus in an 18‐year‐old. Pediatr Dermatol. 2011;28(4):483. []. [PMID:21793895]

Tincopa M, Puttgen KB, Sule S, Cohen BA, Gerstenblith MR. Bullous lupus: an unusual initial presentation of systemic lupus erythematosus in an adolescent girl. Pediatr Dermatol. 2010;27(4):373-6. []. [PMID:20653856]

Yogarajah M, Sivasambu B, Jaffe EA. Bullous systemic lupus erythematosus associated with esophagitis dissecans superficialis. Case Rep Rheumatol. 2015;2015:930683. []. [PMID:25821624]

Contestable JJ, Edhegard KD, Meyerle JH. Bullous systemic lupus erythematosus: a review and update to diagnosis and treatment. Am J Clin Dermatol. 2014;15(6):517-24. []. [PMID:25358414]

Burrows NP, Bhogal BS, Black MM, et al. Bullous eruption of systemic lupus erythematosus: a clinicopathological study of four cases. Br J Dermatol. 1993;128(3):332-8. []. [PMID:8471519]

Vassileva S. Bullous systemic lupus erythematosus. Clin Dermatol. 2004;22(2):129-38. []. [PMID:15234014]

Shirahama S, Furukawa F, Yagi H, Tanaka T, Hashimoto T, Takigawa M. Bullous systemic lupus erythematosus: detection of antibodies against noncollagenous domain of type VII collagen. J Am Acad Dermatol. 1998;38(5 Suppl):844-8. []. [PMID:9591800]

Bain EE, Grover RK, Plunkett RW, Beutner EH. Detection of collagen VII autoantibodies to NC1 and NC2 domains of collagen VII by ELISA in suspected epidermolysis bullosa acquisita and bullous lupus erythematosus patients. J Dermatol Sci. 2012;65(2):155-6. []. [PMID:22225828]

Camisa C, Sharma HM. Vesiculobullous systemic lupus erythematosus: report of two cases and a review of the literature. J Am Acad Dermatol. 1983;9(6):924-33. []. [PMID:6358284]

Camisa C, Grimwood RE. Indirect immunofluorescence in vesiculobullous eruption of systemic lupus erythematosus. J Invest Dermatol. 1986;86(5):606. []. [PMID:3528314]

Camisa C. Vesiculobullous systemic lupus erythematosus: a report of four cases. J Am Acad Dermatol. 1988;18(1 Pt 1):93-100. []. [PMID:3279084]

Piette EW, Werth VP. Dapsone in the management of the autoimmune bullous diseases. Dermatol Clin. 2011;29(4):561-4. []. [PMID:21924998]

Hall RP, Lawley TJ, Smith HR, Katz SI. Bullous eruption of systemic lupus erythematosus. Dramatic response to dapsone therapy. Ann Intern Med. 1982;97(2):165-70. []. [PMID:7049027]

Hans-Bittner NR, Bittner GC, Hans G Fo, Takita LC. Bullous systemic lupus erythematosus in a 10-year-old child. An Bras Dermatol. 2017;92(5 Suppl 1):37-9. []. [PMID:29267441]

Pehr K. Mycophenolate mofetil and erythromycin for bullous lupus erythematosus of childhood. J Cutan Med Surg. 2012;16(1):50-3. []. [PMID:22417996]

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. []. [PMID:12587829]

Alsanafi S, Kovarik C, Mermelstein AL, Werth VP. Rituximab in the treatment of bullous systemic lupus erythematosus. J Clin Rheumatol. 2011;17(3):142-4. []. [PMID:21441817]




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