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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. A rare, chronic, incurable, sub epithelial autoimmune bullous disease characterized by the presence of tissue bound autoantibodies against type VII collagen within the basement membrane zone of the dermal-epidermal junction of stratified squamous epithelia.
The patient's serum may also have anti-type VII collagen autoantibodies. The clinical presentation is varied, and may involve the skin, oral mucosa and the upper third of the esophagus. The classical presentation is reminiscent of hereditary dystrophic epidermolysis bullosa EB with skin fragility, blisters and erosions and skin scarring. Other non-classical clinical presentations include an inflammatory bullous pemphigoid-like eruption, a mucous membrane pemphigoid-like eruption, and an IgA bullous dermatosis-like disease.
Epidermolysis bullosa acquisita EBA is an acquired disease with onset typically in middle-aged adults but can rarely occur in childhood. The disease manifests in several possible ways. The classical presentation is reminiscent of hereditary dystrophic EB with skin fragility, blisters and erosions and skin scarring. There is marked skin fragility with skin blisters and erosions over trauma-prone areas of the skin that results in skin scarring.
This form is relatively non-inflammatory. Mucous membrane lesions and nail dystrophy are common. A second possible clinical presentation is an inflammatory bullous eruption reminiscent of the autoimmune bullous disease, bullous pemphigoid.
These bullae are on inflammatory plaques and may be flexural rather than over trauma-prone areas. A third possible clinical presentation is mucosal-centered with blisters, erosions and scarring of the conjunctival and oral mucosae, but may also involve the larynx and urethra. A fourth possible presentation is an IgA anti-type VII collagen mediated disease reminiscent of IgA bullous dermatosis and dermatitis herpetiformis.
Diseases frequently associated with EBA include inflammatory bowel diseases Crohn's disease and ulcerative colitis and systemic lupus erythematosus. Type VII collagen is the major component of anchoring fibril structures located at the dermal-epidermal junction just beneath the lamina densa.
These structures hold the epidermal and dermal layers of skin together. The autoantibodies against type VII collagen perturbs the function of anchoring fibrils and the adherence between these two main layers of skin is compromised and prone to blistering. In EBA patients without inflammatory bowel disease, oral colchicine is the first-line treatment.
Dapsone may be helpful in some patients. Oral cyclosporine A controls the disease, but its nephrotoxic side-effects limit its use. In the classical form of EBA, other types of immunosuppressive therapy such as systemic steroids, azathioprine, mycophenylate mofetil, are often ineffective, but may be useful in the inflammatory varieties of EBA.
Although further trials are necessary, encouraging results have been obtained with other approaches such as intravenous immunoglobulin therapy, extracorporeal photochemotherapy and, more recently, rituximab therapy.
EBA is a chronic, incurable disease that leads to dystrophic scarring and milia. During the disease course, the inflammatory forms may evolve to resemble the classical form and vice-versa. Although not fatal, the progressive scarring and involvement of the oral and ocular mucosae can be functionally disabling.
Other search option s Alphabetical list. Suggest an update. Summary and related texts. Related genes. Clinical signs. Check this box if you wish to receive a copy of your message. Disease definition A rare, chronic, incurable, sub epithelial autoimmune bullous disease characterized by the presence of tissue bound autoantibodies against type VII collagen within the basement membrane zone of the dermal-epidermal junction of stratified squamous epithelia.
Clinical description Epidermolysis bullosa acquisita EBA is an acquired disease with onset typically in middle-aged adults but can rarely occur in childhood.
Differential diagnosis The differential diagnosis should include other subepidermal, autoimmune bullous diseases. Management and treatment In EBA patients without inflammatory bowel disease, oral colchicine is the first-line treatment.
Prognosis EBA is a chronic, incurable disease that leads to dystrophic scarring and milia. Additional information Further information on this disease Classification s 1 Gene s 0 Clinical signs and symptoms Other website s 4.
Health care resources for this disease Expert centres Diagnostic tests 6 Patient organisations 20 Orphan designation s and orphan drug s 1. Specialised Social Services Eurordis directory. The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.
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Epidermolysis bullosa acquisita: literature review. Ann Dermatol Venereol. Epidermolysis bullosa acquisita: report of three cases and review of all published cases. Arch Dermatol.