Systemic Lupus Erythematosus and Bullous Pemphigoid with Dramatic Response to Dapsone

Abstract

Patient: Female, 11Final Diagnosis: Bullous pemphigoid in systemic lupus erythematosusSymptoms: Bullous lupus • photosensitive rash • synovitisMedication:—Clinical Procedure: Pharmacological treatmentSpecialty: RheumatologyObjective:Unusual clinical courseBackground:Bullous pemphigoid is an autoimmune blistering disease, with relapses, isolated or associated with other autoimmune diseases such as systemic lupus erythematosus (SLE). Joint manifestations rapidly respond to small or moderate doses of corticosteroids, whereas skin manifestations usually respond to antimalarial drugs.Case Report:We describe the clinical case of an 11-year-old girl with SLE. She showed bullous skin lesions with arthralgia, mild proteinuria, resolved after steroid treatment. At the tapering of her prednisone dose, the patient had new skin lesions requiring an increased dose of prednisone. She started dapsone at the dosage of 1 mg/kg/day, maintaining low dose prednisone; this treatment was successfully followed by the dramatic disappearance of skin lesions and limb pain.Conclusions:Bullous skin lesions can represent the first clinical presentation of pediatric SLE and could influence the treatment and the outcome of these patients.This case showed an atypical course as both skin manifestations and arthritis promptly and persistently resolved with dapsone without the use of high-dose glucocorticoids.Only a few cases of patients with SLE associated with bullous pemphigoid have been reported in the literature, and very few in the pediatric population.

Drug-induced Bullous Pemphigoid and Lupus Erythematosus Occurring under Anti-TNF-α and IL-6 Therapy in a Patient with Rheumatoid Arthritis

“…However, after the treatment with biologics, BP developed and she also became complicated with SLE (10,11). Blistering eruptions are extremely rare in RA and SS (12,13), however, bullous diseases including BP may sometimes be associated with SLE (14,15). Moreover, since many drugs can cause druginduced BP and lupus erythematosus (3, 7), our findings suggest that both ADA and TCZ could induce BP and SLE in an RA patient primarily presenting with SS manifestations.…”

Bullous systemic lupus erythematosus a case report

“…The current therapeutic regimens for BSLE are based on the pathogenesis of the disease, case-reports, and case-series. Dapsone is used as first-line therapy6 , 11 , 23 - 25 along with glucocorticoids and immunosuppressants. Other therapeutic options include mycophenolate mofetil5 , 7 , 13 , 26 methotrexate,27 rituximab,28 hydroxychloroquine and intravenous immunoglobulin 8…”

Bullous Systemic Lupus Erythematosus and Membranous Lupus Nephritis in a Filipino Child

“…[16][17][18][19][21][22] BSLE is a rare form of SLE and the absence of pathognomonic clinical features renders the difficulty in clinching the diagnosis. [16][17][18][19]21 The first diagnostic criteria for BSLE was proposed in 1983 by Camisa and Sharma which include the following: (1) a diagnosis of SLE based upon American Rheumatism Association criteria, (2) vesicles and bullae arising upon but not limited to sun-exposed skin, (3) histopathology compatible with dermatitis herpetiformes, (4) Negative indirect immunofluorescence for circulating BMZ Figure 2. Histopathologic examination revealed a sub-epidermal split with neutrophilic infiltrates along the dermo-epidermal junction, moderate perivascular, periadnexal and interstitial infiltrates composed of predominantly neutrophils with neutrophilic dusts, lymphocytes, plasma cells, rare eosinophils and increased dermal mucin.…”

“…To date, there are 21 reported cases in the literature from 1979. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] Of the 357 cases of juvenile SLE and 169 cases of childhood lupus nephritis seen by the section of Pediatric Rheumatology at the Philippine General Hospital (PGH) since 2005, this is the first documented case that presented with generalized vesiculobullous lesions. 20…”