Naslov
Commentary: Supepidermal Bullous Skin Diseases

Autori
Lipozenčić, Jasna ; Marinović, Branka

Izvornik
Clinics in dermatology (0738-081X) 30 (2012), 1; 1-2

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, uvodnik, ostalo

Ključne riječi
Bullous pemphigoid; subepidermal bullous skin diseases; therapies

Sažetak
Bullous pemphigoid (BP) group is subepidermal autoimmune blistering diseases with three clinical variants of this disease: BP, cicatricial pemphigoid (CP) and pemphigoid gestationis (PG). The autoimmune response in BP is directed against two hemidesmosomal antigenes, BP 180 (BP antigen 2 or collagen XVII) and BP 230 (BP antigen 1). In CP are included multiple antigens: BP 180, BP 230, laminin-5, laminin-6 and integrin B4 subunit. Autoantibodies in PG are mainly reactive with BP 180. Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering skin disease in which are included autoantibody to type VII collagen (290kd autoantigen), a component of the anchoring fibril complex of the basement membrane zone. There are a clinical presentations of this disease: the classic porphyria cutanea tarda-like ; non-inflammatory mechanobullous disease ; the bullous pemphigoid presentation with widespread blisters ; the cicatricial pemphigoid-like form with mucous membrane involvement ; the Brunsting-Perri-like presentation (head and neck area - localized form) ; the immunoglobulin A (IgA) bullous dermatosis-like form with neutrophil rich infiltrate. EBA may be associated with underlying diseases such as inflammatory bowel disease, systemic lupus erythematosus, amyloidosis and other inflammatory and autoimmune conditions. Dermatitis herpetiformis belongs to IgA dermatoses diverse group of disease in which process occurs directly from IgA in the lesional or perilesional skin. In this group are: Linear IgA bullous dermatoses/chronic bullous disease of childhood ; IgA pemphigus: subcorneal pustular dermatosis type ; IgA pemphigus: intraepidermal type and IgA vasculitis (Henoch-Schönlein purpura). This group of diseases has in common the presence of a neutrophil-rich inflammatory infiltrate and response to dapson therapy. Neutrophils express IgA receptors (FcαR1). In BP effective therapy can be achieved with topical high potency corticosteroids, with systemic corticosteroids, azathioprine, cyclosporine A and mycophenolate mofetil, dapsone, plasmapheresis, IVIG, tetracycline and nicotinamide for control of BP with limited effect. In refractory EBA colchicine, systemic steroids, mycophenolic acid, azathioprine, methotrexate, cyclosporin A, cyclophosphamide, and gamma globuline can be helpful in controlling EBA when it appears as in inflammatory BP-like disease. The anti-tumor necrosis factor-alpha (TNF-α) biologics have been tried in EBA with some success. Supportive therapy is warranted in all patients with EBA. In DH patients in without methoglobinemia first line therapy is with dapsone, and sulfapyridine. For successful treatment of pemphigus, one must reduce autoantibody production, while blocking any of the several steps in the inflammatory cascade can treat bullous pemphigoid. Keeping the patient alive by suppressing disease, maintaining the quality of life, and minimizing drug side effects are major goals for the treatment of bullous skin diseases. When EBA is diagnosed, it is imperative to consider complications of disease and association with other inflammatory conditions that are frequently associated with EBA. Immunosuppressive therapy is usually required. Because neutrophils are important effector cells that mediate tissue damage in IgA dermatoses, responsiveness exhibit to sulfone therapy. This important scientific filed of bullous skin diseases is classified in issue two.

Izvorni jezik
Engleski

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