Epidermolysis bullosa acquisita with moderately severe dysphagia due to esophageal strictures

Abstract

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.

Hand eczema

“…In an open label study, Mittal et al . [ 98 ] used methotrexate 10-15 mg weekly in single or divided doses for 12 weeks in 15 patients of recalcitrant eczema (patients with pompholyx, n = 6), and found that two patients of pompholyx had excellent response (almost complete remission) and one patient of pompholyx had good response (partial remission). The main effect of methotrexate is on epidermal proliferation by its metabolite polyglutamate causing adenosine release.…”

Japanese guidelines for the management of pemphigoid (including epidermolysis bullosa acquisita)

“…Explanation : Tu et al . reported that improvement was achieved by administrating prednisolone (50 mg/day) and azathioprine (150 mg/day) to patients with treatment‐resistant EBA with severe mucosal lesions, and it was possible to reduce the dose to prednisolone 5 mg/day . Ito et al .…”

Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita - Brazilian Society of Dermatology

“…Azathioprine has varying results in the literature. 101,104,113 Although its use is well established in pemphigus vulgaris, its application in EBA is less convincing, with other medications, such as dapsone and mycophenolate mofetil, being preferred more often. 114…”

“…139,140 Esophageal involvement may present as the formation of erosions and stenosis and dysphagia. 113,141 Sometimes, patients must undergo periodic esophageal dilations and, in extreme cases, gastrostomy.…”