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Epidermolysis bullosa acquisita with moderately severe Dysphagia due to esophageal strictures.

Tu J, Kumarasinghe PW - Indian J Dermatol (2011)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Sir Charles Gairdner Hospital, Nedlands, Western Australia 6009, Australia.

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.

No MeSH data available.


Related in: MedlinePlus

Subepidermal vesicle (H and E, ×40)
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Figure 3: Subepidermal vesicle (H and E, ×40)

Mentions: On examination, multiple vesicles and bullae were present over the hands, feet, upper and lower limbs and in the mouth. There was a predilection for sites with friction, including the sole of the foot, medial malleolus, elbows and finger tips [Figures 1 and 2]. A biopsy was taken of a new lesion on the patient's right wrist and sent for histopathology and immunofluorescence. Results demonstrated separation of the epidermis from the dermis, with formation of a subepidermal vesicle [Figure 3]. Fibrinous material and a mixed lymphocytic and neutrophilic infiltrate were seen with no significant eosinophilia. Direct immunofluorescence showed intense linear junctional IgG and C3 at the dermo-epidermal junction, localized to the dermal aspect of the dermo-epidermal split. Serological tests for circulating anti-basement membrane (junctional) autoantibodies were significantly positive (2 in a scale of 0–3). Intercellular antibodies were negative. These findings were consistent with a diagnosis of EBA.


Epidermolysis bullosa acquisita with moderately severe Dysphagia due to esophageal strictures.

Tu J, Kumarasinghe PW - Indian J Dermatol (2011)

Subepidermal vesicle (H and E, ×40)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3108531&req=5

Figure 3: Subepidermal vesicle (H and E, ×40)
Mentions: On examination, multiple vesicles and bullae were present over the hands, feet, upper and lower limbs and in the mouth. There was a predilection for sites with friction, including the sole of the foot, medial malleolus, elbows and finger tips [Figures 1 and 2]. A biopsy was taken of a new lesion on the patient's right wrist and sent for histopathology and immunofluorescence. Results demonstrated separation of the epidermis from the dermis, with formation of a subepidermal vesicle [Figure 3]. Fibrinous material and a mixed lymphocytic and neutrophilic infiltrate were seen with no significant eosinophilia. Direct immunofluorescence showed intense linear junctional IgG and C3 at the dermo-epidermal junction, localized to the dermal aspect of the dermo-epidermal split. Serological tests for circulating anti-basement membrane (junctional) autoantibodies were significantly positive (2 in a scale of 0–3). Intercellular antibodies were negative. These findings were consistent with a diagnosis of EBA.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Sir Charles Gairdner Hospital, Nedlands, Western Australia 6009, Australia.

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.

No MeSH data available.


Related in: MedlinePlus