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.
WatanabeT, TsuchidaT. Classification of lupus erythematosus based upon cutaneous manifestations. Dermatological, systemic and laboratory findings in 191 patients. Dermatology. 1995;190(4):277-83. [https://doi.org/10.1159/000246716]. [PMID:7655105]
NicoMMS, LourençoSV. Multiple Blisters Along the Lip Vermilion are a Clue to Bullous Lupus Erythematosus. Acta Derm Venereol. 2012;92(4):404-5. [https://doi.org/10.2340/00015555-1276]. [PMID:22293956]
LiAW, WeedJG, StameyCR, SubtilA, TomaykoMM, AntayaRJ. Bullous systemic lupus erythematosus in a 6‐year‐old boy. Pediatr Dermatol. 2017;34(6):e313-6. [https://doi.org/10.1111/pde.13258]. [PMID:28851077]
MaggioMC, CorselloG, PrinziE, CimazR. Systemic lupus erythematosus and bullous pemphigoid with dramatic response to dapsone. Am J Case Rep. 2017;18:317-9. [https://doi.org/10.12659/AJCR.902351]. [PMID:28352068]
MaheshE, MadhyasthaPR, VarmaV, GurudevKC, GireeshMS, BandeSR. Unusual presentation of systemic lupus erythematosus. Saudi J Kidney Dis Transpl. 2017;28(3):653-6. [https://doi.org/10.4103/1319-2442.206468]. [PMID:28540909]
JuhászM, RoggeM, ChenM, CzernikA, KimSJ, GellerL. Case of pediatric bullous systemic lupus erythematosus treated with intravenous immunoglobulin. Pediatr Dermatol. 2017;34(1):e54-6. [https://doi.org/10.1111/pde.13015]. [PMID:27778386]
GrabellDA, MatthewsLA, YanceyKB, ChongBF. Detection of type VII collagen autoantibodies before the onset of bullous systemic lupus erythematosus. JAMA Dermatol. 2015;151(5):539-43. [https://doi.org/10.1001/jamadermatol.2014.4409]. [PMID:25671758]
ChristodoulouG, PowellM, NguyenV, WattersK, JafarianF. An atypical case of bullous systemic lupus erythematosus in a 16‐year‐old boy. Pediatr Dermatol. 2014;31(6):e164-6. [https://doi.org/10.1111/pde.12389]. [PMID:25040539]
LiuKL, ShenJ, YangC, ChenYJ. Bullous systemic lupus erythematosus in a child responding to Dapsone. Pediatr Dermatol. 2014;31(4):e104-6. [https://doi.org/10.1111/pde.12340]. [PMID:24846529]
TincopaM, PuttgenKB, SuleS, CohenBA, GerstenblithMR. Bullous lupus: an unusual initial presentation of systemic lupus erythematosus in an adolescent girl. Pediatr Dermatol. 2010;27(4):373-6. [https://doi.org/10.1111/j.1525-1470.2010.01179.x]. [PMID:20653856]
YogarajahM, SivasambuB, JaffeEA. Bullous systemic lupus erythematosus associated with esophagitis dissecans superficialis. Case Rep Rheumatol. 2015;2015:930683. [https://doi.org/10.1155/2015/930683]. [PMID:25821624]
ContestableJJ, EdhegardKD, MeyerleJH. Bullous systemic lupus erythematosus: a review and update to diagnosis and treatment. Am J Clin Dermatol. 2014;15(6):517-24. [https://doi.org/10.1007/s40257-014-0098-0]. [PMID:25358414]
ShirahamaS, FurukawaF, YagiH, TanakaT, HashimotoT, TakigawaM. Bullous systemic lupus erythematosus: detection of antibodies against noncollagenous domain of type VII collagen. J Am Acad Dermatol. 1998;38(5Suppl):844-8. [https://doi.org/10.1016/S0190-9622(98)70472-4]. [PMID:9591800]
BainEE, GroverRK, PlunkettRW, BeutnerEH. 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. [https://doi.org/10.1016/j.jdermsci.2011.12.004]. [PMID:22225828]
Facebook
Google+
Twitter
LinkedIn
Mendeley
StumbleUpon
CiteULike
Reddit
Email