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At a glance

Group of well-defined chronic nonhereditary blistering autoimmune disorders. Characterized by autoantibodies against structural proteins of the dermal-epidermal junction. Tense blisters and erosions occur on skin or mucous membranes close to the skin surface.

Synonyms

Bullous Pemphigoid Brunsting-Perry Type; Cicatricial pemphigoid; Mucous Membrane Pemphigoid; Pemphigoid Gestationis; Linear IgA Disease; Epidermis Bullosa Acquisita; Lichen Planus Pemphigoides.

Incidence

Estimated to be 6 to 20:1,000,000 per year with an increasing incidence worldwide.

Classification

At least seven separate disorders have been identified:

  • Bullous Pemphigoid: Affects uniquely the skin.

  • Mucus Membrane Pemphigoid (Cicatricial Pemphigoid): Affects the mucosal surfaces of the body and, occasionally, the skin.

  • Pemphigoid Gestationis: Manifests during pregnancy.

  • Linear IgA Disease: Linear binding of IgA at the dermal-epidermal junction and clinical overlap with bullous pemphigoid.

  • Epidermiolysis Bullosa Acquisita (Epidermis Bullosa Acquisita): Chronic autoimmune subepidermal blistering disease, involving the skin and the mucus membrane.

  • Lichen Planus Pemphigoid: Chronic inflammatory and immune medicated disease that affects the skin, nails, hair and mucous membrane.

  • Anti-p200/Anti-Laminin γ1 Pemphigoid

Pathophysiology

Autoantibodies are formed against distinct structural components of the dermal-epidermal junction. The junction proteins link the cytoskeleton of the basal keratinocytes to the extracellular matrix of the dermis. The binding of pemphigoid autoantibodies (P180, BP230, laminin 332, LAD-1, Type IV collagen, Laminin gamma1) lead to the separation of the epidermis and dermis.

Diagnosis

Clinical appearance of the eruption and skin biopsy.

Clinical aspects

  • Bullous Pemphigoid: Multiple tense bullae arising from large, irregular urticarial plaques; lesions occur most typically on the flexion surfaces of the extremities or lower torso but may be generalized in severe cases. It may occur at any age, but is most common in the elderly. Clinical course of exacerbations and remissions. Treatment is effective with very potent topical steroids. The use of steroids in widespread disease might be limited by side effects.

  • Mucous Membrane Pemphigoid: Bullous eruptions involving mainly the oropharynx, conjunctiva, larynx, esophagus, genitalia, and anus. The scarring of the mucosal surface as a consequence of the recurrent blistering can result in blindness, nasal or laryngeal obstruction, or esophageal stenosis. It is often associated with dermatitis herpetiformis, also with multiple sclerosis (with no involvement of mucous membrane, but with an early onset). It is described as associated with autoimmune thrombocytopenia, nephropathy, and ulcerative colitis.

Precautions before anesthesia

History of the associated disease: renal, ulcerative colitis, multiple sclerosis. History of medications, usually high-dose corticosteroids, dapsone, azathioprine, and/or other immunosuppressive agents such as cyclosporin.

Anesthetic considerations

The same considerations as for the other blistering diseases: difficult venous access, friction of the skin or mucosae may cause blistering; careful padding; avoid tapes; use petroleum jelly gauze; lubricate blade and ...

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