Epidermolysis bullosa acquisita, BP-like EBA L12.-

The BP-like epidermolysis bullosa acquisita variant is a rare, acquired, blistering autoimmune disease with IgG antibodies against type VII collagen (NC1 domain), the main component of the "anchoring fibrils" in the dermoepidermal junction zone. The disease can occur after viral infections, as a paraneoplastic syndrome or after autoimmunological reactions (e.g. after autologous stem cell transplantation).

Based on their clinical appearance, two main subgroups of epidemolysiosis-acquisita diseases (EBA) can be distinguished:

• classical or mechanobullous EBA (non-inflammatory) and
• non-classical inflammatory or non-mechanobullous EBA which is subdivided as follows:
  • Epidermolysis bullosa acquisita, bullous pemphigoid-like EBA (47%) - tight blisters on erythematous or urticarial skin, mainly trunk and extremities
  • Epidermolysis bullosa acquisita, mucosal pemphigoid-like EBA (7%) - lesions and scarring on the mucous membranes of the mouth, oesophagus, trachea and esophagus. oesophagus, trachea, conjunctiva
  • Epidermolysis bullosa acquisita, scarring mucosal pemphigoid-like EBA (7%) - variant under the picture of a localized scarring pemphigoid; chronic recurrent bullous dermatosis on the head and neck, sometimes with hair loss, usually no mucosal involvement
  • Epidermolysis bullosa acquisita, linear IgA dermatosis-like EBA/IgA-EBA (3%) - variant under the picture of a linear IgA dermatosis of childhood. Linear IgA deposits in the basement membrane zone

In the inflammatory variants, skin changes occur all over the skin, not just in the areas most frequently exposed to trauma. Therefore, it may resemble other subepidermal autoimmune bullous dermatoses, such as bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA bullous dermatosis and Brunsting-Perry pemphigoid. The occurrence of scars and milia during the course of the disease is rarer than in mechanobullous EBA.

Bullous pemphigoid (BP)-like EBA: This variant is the most common subtype of all EBA, accounting for around 50%, and presents with tense vesiculobullous lesions on urticarial, erythematous, pruritic plaques on all skin sites, including the face: the oral mucosa may also be affected. The clinical picture cannot be distinguished from bullous pemphigoid both clinically and in the laboratory, as both show subepidermal blisters on histopathologic examination, while direct immunofluorescence shows linear deposition of C3 and IgG in the basement membrane zone. Patients sometimes also present with lesions suggestive of mechanobullous EBA. The lesions can lead to atrophic scars and milia after healing, although these occur less frequently than in the classic form.

• Oral, intravenous or corticosteroid pulse therapies are considered the first choice for the treatment of EBA. The exact mechanism of action of corticosteroids in EBA is not yet fully understood. The recommended starting dose ranges from 0.5 mg/kg/day in mild cases to 1.5 mg/kg/day in severe cases.
• In patients with dysphagia, oral corticosteroid solutions may be more effective and better tolerated than tablets. Immunosuppressants, immunomodulators and rituximab are also used if necessary.
• Dapsone (DDS): DDS in a dose of 25-150 mg/day reduces neutrophil chemotaxis and has a corticosteroid-sparing effect. DDS can even be used in monotherapy with adequate control of IgA-mediated EBA activity and in pediatric patients. A response to treatment is usually observed within 2 weeks of starting treatment. Determination of glucose-6-phosphate dehydrogenase levels is also recommended as low levels correlate with hemolysis.
• Colchicine: Mild MSD can be controlled with colchicine 1-2 mg/day alone or in combination with systemic corticosteroid therapy, with few side effects such as dose-dependent diarrhea. Colchicine is beneficial in patients in whom immunosuppressants should be avoided as it inhibits neutrophil chemotaxis and increases prostaglandin E2.

Alternative:

• Cyclosporine (CyA): CyA at a dose of 4 to 9 mg/kg/day has rarely been used as adjuvant treatment for EBA.
• Mycophenolate mofetil (MMF): MMF at a dose of 2 to 3 g/day has been successfully used in combination with systemic corticosteroid therapy to promote remission of the disease and even as monotherapy after complete corticosteroid discontinuation.
• Azathioprine (AZA): AZA at a dose of 2 to 3 mg/kg/day can be combined with systemic corticosteroid therapy in moderate to severe MSD. The immunosuppressive effect includes inhibition of nucleic acid and protein synthesis with a reduction in mononuclear cells and lymphocytes.

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