Introduction

Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune subepidermal blistering disease. Elliot first reported a patient with a non-hereditary form of epidermolysis bullosa in 1895, and Kablitz first coined the term ‘epidermolysis bullosa acquisita’.

History

The first information of a patient with a bullous disease reminiscent of epidermolysis bullosa (EB) with no known affected family members was reported by Elliot in 1895.

In the 1980s, Yaoita et al. and Nieboer et al. found that although EBA and BP exhibit IgG deposits at the dermal–epidermal junction (DEJ) by direct immunofluorescence (DIF), the diseases could be distinguished by immunoelectron microscopy (IEM).

In 1984, Woodley et al. identified the antigenic target for EBA antibodies as a 290 kDa collagenous protein within the DEJ of human skin and later showed that this protein was C7 within AFs.

Etiology

The aetiology is unknown, it is hypothesised that EBA is most likely an autoimmune phenomenon because the disease involves IgG autoantibodies directed against C7.

Epidemiology

EBA is an uncommon, autoimmune bullous disease with a commonness of around 0.2/million people.

EBA does not favour any race or gender, yet it has recently been suggested that the Korean population has a higher incidence of EBA. The age of onset varies widely from early childhood to late adult life, but most cases begin between the fourth and the fifth decades.

Pathogenesis

EBA is characterised by IgG autoantibodies directly attacking type VII

Collagen within AFs (anchoring fibrils), subsequently leading to a shortage of normal AFs. The resulting lack of AFs causes dissociation of the epidermis and BMZ (Basement membrane zone) from the underlying papillary dermis. IgG autoantibodies can be visualised at the DEJ (Dermal–epidermal junction), which confirms the likelihood that this disease is an autoimmune process.

Classical presentation

Classical EBA is a mechanobullous disease marked by skin fragility over trauma-prone surfaces. These patients typically present with blisters, erosions, and scars over the backs of the hands, elbows, knees, sacral area, and

feet.

Effect in oral mucosa is normally significant, with erosions and frank blisters.

They can be haemorrhagic and can result in erosions, crusts, scales, scars, scarring, alopecia, milia cysts, and nail dystrophy. The lesions heal with scarring and frequently with the formation of pearl-like milia cysts within the scarred areas.

In addition to the mechanobullous classic variant, several inflammatory subtypes of EBA were described, clinically mimicking bullous pemphigoid,

linear IgA disease, mucous membrane pemphigoid or Brunsting–Perry pemphigoid.

Clinical appearance of epidermolysis bullosa acquisita (EBA). Skin fragility and tense blisters on the heel and ankles, located primarily on anatomic areas subjected to repetitive minor trauma (a, b). Erosive lesions on the back and buttock (c). Blisters and erosions with erythematous atrophic plaques on the neck (d) and face (e). EBA also presents with mucosal involvements (f). The lesions heal with scar formation (g), scarring alopecia (h) and formation of milia (i), which follows blisters.

Diagnosis

The criteria for the diagnosis of EBA have been established with a few slight modifications:

1. A bullous disease within defined clinical scope
2. Little information of family history of a bullous disease
3. IEM of perilesional skin indicating localization of IgG deposits within the lower lamina densa and/or sub lamina densa zone of the DEJ.
4. Another laboratory examination for item 5 include indirect or direct SSSI, IIF using substrate that is lacking with basement membrane molecules, Western blotting,

Management

1.     The first-line drug for EBA therapy is colchicine. More shots/doses of colchicine have been informed to be effective in patients with both classical and inflammatory clinical manifestations of EBA. Colchicine has minimum side effects as compared to other medications.
2.     Another drug for consideration in EBA therapy is the immunosuppressant cyclosporine.
3.     Other immunosuppressants may also be tried, including methotrexate, azathioprine, and cyclophosphamide. Dapsone and prednisone can help some EBA patients, specifically those with neutrophils in the dermal infiltrate. Studies have shown that EBA patients with the classical mechanobullous representation are normally refractory to systemic glucocorticoids.

“As with many life-altering events, an autoimmune illness is almost guaranteed to cause you to re-evaluate your priorities.”

― Joan Friedlander, Women, Work, and Autoimmune Disease

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