Epidermolysis bullosa acquisita, MM-EBA (7%)

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

It is assumed that EBA has a genetic predisposition, with an overrepresentation of HLA-DRB1*15 and HLA-DRB1*16, whereby there is a connection with the allele HLA-DRB1*15:03, particularly in patients of African descent. The autoantigen is collagen type VII, which is the main component of the anchoring fibrils of the papillary dermis. The antibodies bind to the NC1 domain of collagen type VII (see below Epidermolysis bullosa acquisita/Overview)

Inflammatory forms of EBA: In the inflammatory form, skin lesions 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 dermatosis.

The mucous membrane pemphigoid-like EBA variant mainly affects the mucous membranes, such as the mouth, throat, oesophagus, conjunctiva, anus, genital area and respiratory tract (trachea and bronchi). The scarring (atrophic scars, synechiae and stenoses) are identical to those that occur in the EBA type Brunsting-Perry. They can lead to significant dysfunction of the esophagus, larynx, trachea, bronchi and conjunctiva, which can result in symblepharon, trichiasis and even loss of vision. Esophageal stenosis usually occurs in the upper part and leads to dysphagia, weight loss, malnutrition and even lung infections due to aspiration of food. Larger lesions in the airways can lead to perforation of the nasal septum, pharyngeal and laryngeal stenosis and, more rarely, tracheal and bronchial stenosis, which can result in asphyxia.

see below Epidermolysis bullosa acquisita/Overview

Preventing new lesions involves educating patients about the particularities of this disease. It is important to avoid foods that can cause additional damage to the oral mucosa when chewing and swallowing, such as hot drinks and acidic, rough/crunchy products.

Sequelae may require surgical treatment, including oculoplastic surgery, removal of nasal, urethral and gynecologic synechiae, esophageal dilation or gastrostomy for severe stenosis, and tracheostomy for laryngeal involvement with luminal narrowing and airway obstruction.

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