Subcutaneous immunoglobulin therapy for immunomodulation in a patient with severe epidermolysis bullosa acquisita

Tayal, U.; Burton, Janet; Dash, C. H.; Wojnarowska, Fenella; Chapel, H M

doi:10.1016/j.clim.2008.08.003

Cited by 21 publications

(16 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Intravenous immunoglobulin has also been reported to be used in 15 other EBA patients in the English Literature. [15][16][17][18][19][20][21][22][23][24][25] (Table 3) Majority of these patients had significant, wide spread and severe disease. Twelve of these patients had mucocutaneous involvement, 15,[17][18][19][20][22][23][24] while cutaneous involvement and mucosal involvement only was reported in one patient each.…”

Section: Discussionmentioning

confidence: 99%

Treatment of epidermolysis bullosa acquisita with intravenous immunoglobulin in patients non‐responsive to conventional therapy: clinical outcome and post‐treatment long‐term follow‐up

Ahmed¹,

Gürcan²

2011

Acad Dermatol Venereol

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

Treatment of epidermolysis bullosa acquisita with intravenous immunoglobulin in patients non‐responsive to conventional therapy: clinical outcome and post‐treatment long‐term follow‐up

Ahmed¹,

Gürcan²

2011

Acad Dermatol Venereol

View full text Add to dashboard Cite

show abstract

“…[12–14] More recently, subcutaneous immunoglobulin therapy has shown promise as maintenance therapy, particularly benefiting patients with difficult venous access and providing the option of home therapy. [15]…”

Section: Discussionmentioning

confidence: 99%

Epidermolysis bullosa acquisita with moderately severe dysphagia due to esophageal strictures

Kumarasinghe

2011

Indian J Dermatol

View full text Add to dashboard Cite

Epidermolysis bullosa acquisita (EBA) is a chronic, autoimmune condition involving the skin and mucous membranes. Symptomatic mucosal involvement is rare, but can impact on quality of life, due to esophageal strictures and dysphagia. We report a case involving a 60-year-old male presenting with bullous skin lesions on areas of friction on his hands, feet and mouth. Milia were visible on some healed areas. Biopsy showed a subepidermal vesicle. Direct immunofluorescence showed intense linear junctional IgG and C3 at the dermo-epidermal junction. Serological tests also supported the diagnosis of EBA. Screening tests for underlying malignancies were negative. Despite treatment with systemic steroids, the patient developed increasing dysphagia, requiring further investigation with esophagoscopy and a barium swallow. Confirmation of extensive esophageal stricturing prompted adjustment of medications including an increase in systemic steroids and addition of azathioprine. Currently, the patient's disease remains under control, with improvement in all his symptoms and return of anti-basement membrane antibody levels to normal, whilst he remains on azathioprine 150 mg daily and prednisolone 5 mg daily. This case highlights the fact that the treatment of a given patient with EBA depends on severity of disease and co-morbid symptoms. Newer immunoglobulin and biological therapies have shown promise in treatment resistant disease. Considering that long-term immunosuppressants or biologicals will be required, potential side effects of the drugs should be considered. If further deterioration occurs in this patient, cyclosporin A or intravenous immunoglobulin (IV Ig) will be considered. Vigilance for associated co-morbidities, especially malignancies, should always be maintained.

show abstract

“…The patient's disease was difficult to control and finally responded to subcutaneous immunoglobulin (SCIg) treatment. 2 However, 9 years later, while being treated with prednisolone 8 mg and SCIg, the patient developed clinically diagnosed oral LP, which to date has not responded to the SCIg treatment.…”

Section: Reportmentioning

confidence: 99%

Autoreactivity to bullous pemphigoid 180: is this the link between subepidermal blistering diseases and oral lichen planus?

Shipman

Cooper

Wojnarowska

2011

Clinical and Experimental Dermatology

Self Cite

View full text Add to dashboard Cite

Subepidermal blistering diseases are antibody-mediated diseases. The antigens differ between disease type, but bullous pemphigoid (BP)180 (collagen XVII) is a common finding in several clinical conditions. We report four patients with autoimmune blistering disease [linear Ig A disease (n=2), bullous pemphigoid (n=1), and epidermolysis bullosa acquisita (n=1)], all of whom also developed oral lichen planus (LP). In all cases, the diagnosis of the blistering disease predated the development of the oral lesions. All patients were under the care of dermatologists and of oral medicine/surgery doctors for the diagnosis and management of the oral LP. All had circulating antibodies to BP180 protein. To our knowledge, the association between blistering diseases and oral LP has not been reported previously, and may reflect autoimmunity to BP180.

show abstract

Subcutaneous immunoglobulin therapy for immunomodulation in a patient with severe epidermolysis bullosa acquisita

Cited by 21 publications

References 7 publications

Treatment of epidermolysis bullosa acquisita with intravenous immunoglobulin in patients non‐responsive to conventional therapy: clinical outcome and post‐treatment long‐term follow‐up

Treatment of epidermolysis bullosa acquisita with intravenous immunoglobulin in patients non‐responsive to conventional therapy: clinical outcome and post‐treatment long‐term follow‐up

Epidermolysis bullosa acquisita with moderately severe dysphagia due to esophageal strictures

Autoreactivity to bullous pemphigoid 180: is this the link between subepidermal blistering diseases and oral lichen planus?

Contact Info

Product

Resources

About