Bullous pemphigoid is the most frequent autoimmune blistering skin disease of the elderly. It is mediated by circulating antibodies directed against two hemidesmosomal proteins of the dermal epidermal junction: BPAG1 and BPAG2. Clinical features consist of pruritus and tense blisters usually surrounded by erythema. Blisters sometimes evolve to erosions, become haemorrhagic or even large erosive areas. Lesions heal without scarring. Lesions are symmetrically located on the thighs, legs, trunck and arms. Mucous membranes are usually uninvolved. Histological examination of a skin biopsy specimen shows a subepidermal blister with eosinophils within the blister and the superficial dermis. Direct immunofluorescence shows linear IgG and/or C3 deposits along the dermal epidermal junction. In France and in Europe, most patients are now treated using topical steroid therapy (clobetasol propionate).
J Dermatol Sci. 2014. Update on the pathogenesis of bullous pemphigoid: An autoantibody-mediated blistering disease targeting collagen XVII. BP is a common autoimmune blistering skin disorder that tends to affect the elderly. Autoantibodies (autoAbs) from BP patients react with two hemidesmosomal components: transmembrane collagen XVII and plakin family protein BP230. Of these, collagen XVII (COL17) is thought to be a major autoantigen. The binding of autoAbs to COL17 following the activation of complements and inflammatory pathways eventually leads to the degradation of COL17, and this has been regarded as the main pathogenesis of BP. However, recent investigations have suggested other pathways, including a complement-independent pathway and a pathway involving IgE-autoAbs. BP-autoAbs can directly deplete COL17, leading to fragility of the dermal-epidermal junction. In addition, IgE-autoAbs to COL17 may be involved in the formation of itchy urticarial erythema associated with eosinophilic infiltration.
J Dermatol. 2015. Bullous pemphigoid: What's ahead? Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disease mainly affecting older individuals. Pathogenic autoantibodies preferentially target the non-collagenous 16A domain of collagen XVII (also called BP antigen 2, BPAG2) present in hemidesmosomes. The pathogenic anti-BPAG2 antibodies cause the dermal-epidermal separation in neonatal and adult mice as well as in cryosections of human skin. These experimental BP models stress a pivotal role for neutrophils and the Fcγ receptor of immunoglobulins. Mice that have been genetically manipulated in the pathogenic domain of BPAG2 spontaneously develop subepidermal blistering with pruritus and eosinophilic infiltration. BPAG2 is physiologically and aberrantly expressed in neuronal tissue and internal malignancies, and the associations of BP with Parkinson's disease, stroke and internal malignancies invites new investigations into the immunological dysregulation behind the comorbidity.
Cicatricial pemphigoid information and treatment
Cicatricial pemphigoid is an eye disorder. The ocular manifestations of autoimmune bullous diseases are common and potentially sight-threatening. Major ophthalmic involvement is most commonly seen in mucous membrane pemphigoid (cicatricial pemphigoid), epidermolysis bullosa acquisita, linear IgA bullous disease, pemphigus vulgaris and paraneoplastic pemphigus. The main pathological process is related to autoimmune-induced conjunctival inflammation with consequent lid and corneal pathology, which may eventually result in permanent visual loss. Ocular involvement can be asymptomatic. Early detection is aided by careful attention to symptoms and signs of early ophthalmic disease. Ocular disease can be difficult to treat and management usually involves systemic therapy with immunomodulators to control inflammation and prevent progression to irreversible blindness, as well as surgical intervention in advanced disease.
I was first diagnosed with Bullous Pemphagoid in 2011.
There are no more sores or blisters, but the rash and itch, persist.. I am
looking for a natural alternative remedy for the skin rash and itch (as I am
allergic to all Steroid prescription drugs).
A. As of 2017 I am not aware yet of natural remedies.
Someone on your website asked for anything that helps to relieve the itch and redness of bullous pemphigoid. They might try EumuaidMax an essential oil/emu oil salve that has helped me. It hasnít cured it, but it is much improved even without continued use of steroids. Also low dose naltrexone might help. It got me off the prednisone.