the-goeckerman-regimen-for-treatment-moderate-to-severe

The Goeckerman Regimen for the Treatment of Moderate to Severe Psoriasis

Psoriasis is a  chronic, immune-mediated inflammatory skin disease affecting approximately 2-3%  of the population. The Goeckerman regimen consists of ...

University of Southern California

University of California, San Francisco 

Epidermolysis bullosa acquisita (EBA) is a rare, acquired, chronic subepidermal bullous disease of the skin and mucosa characterized by autoantibodies to type VII collagen (C7) structures, a major component of anchoring fibrils, which attach the epidermis to the dermis. EBA patients have tissue-bound and circulating antitype C7 autoantibodies that attack type C7 and result in a reduction or perturbation of normally functioning anchoring fibrils. Patients with EBA have skin fragility, blisters, erosions, scars, milia, and nail loss, all features reminiscent of genetic dystrophic epidermolysis bullosa. These immunoglobulin G antitype C7 antibodies are pathogenic, because when they are injected into mice, the mice develop an EBA-like blistering disease. In addition to the classical mechanobullous presentation, EBA also has several other distinct clinical syndromes similar to bullous pemphigoid, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. Although treatment for EBA is often unsatisfactory, some therapeutic success has been achieved with colchicine, dapsone, plasmapheresis, photopheresis, infliximab, and intravenous immunoglobulin.

Epidermolysis bullosa acquisita.

Epidermolyse bulleuse acquise (EBA) est une maladie bulleuse sous-&eacute;pidermique rare, acquise, chronique de la peau et des muqueuses caract&eacute;ris&eacute;e par des auto-anticorps aux structures de type VII collag&egrave;ne (C7), une composante majeure de l&#39;ancrage des fibrilles, qui attachent l&#39;&eacute;piderme dans le derme. Tissu li&eacute; ont les patients EBA et circulant antitype C7 les auto-anticorps qui attaquent type C7 et normalement entra&icirc;nent une r&eacute;duction ou une perturbation de fonctionnement de fibrilles d&#39;ancrage. Patients atteints de TSA ont la fragilit&eacute; cutan&eacute;e, cloques, &eacute;rosions, cicatrices, milia et perte de l&#39;ongle, toutes les fonctionnalit&eacute;s de g&eacute;n&eacute;tique de l&#39;&eacute;pidermolyse bulleuse dystrophique. Ces anticorps C7 de l&#39;immunoglobuline G antitype sont pathog&egrave;nes, car lorsqu&#39;elles sont inject&eacute;es &agrave; des souris, les souris d&eacute;veloppent une maladie de cloques EBA semblable. Outre la pr&eacute;sentation classique mechanobullous, Abe a &eacute;galement o&ugrave; sont indiqu&eacute;es plusieurs autres syndromes cliniques distinctes semblables &agrave; la Pemphigo&iuml;de bulleuse, Pemphigo&iuml;de Brunsting-Perry ou Pemphigo&iuml;de cicatricielle. Bien que le traitement pour EBA n&#39;est souvent pas satisfaisant, certains succ&egrave;s th&eacute;rapeutique a &eacute;t&eacute; atteint avec la colchicine, la dapsone, plasmaph&eacute;r&egrave;se, photopheresis, infliximab et immunoglobulines par voie intraveineuse.

Epidermolyse bulleuse acquise.

Epidermolisi bollosa acquisita (EBA) &egrave; una malattia bollosa subepidermica rara, acquisite, croniche della cute e della mucosa caratterizzata da autoanticorpi di tipo VII strutture collagene (C7), una componente importante di ancoraggio fibrille, che fissano l&#39;epidermide al derma. I pazienti EBA hanno associato a tessuto e circolanti battesimo C7 autoanticorpi che attaccano tipo C7 e causare una riduzione o perturbazione di normalmente funzionamento fibrille di ancoraggio. I pazienti con EBA hanno fragilit&agrave; cutanea, vescicole, erosioni, cicatrici, milia e perdita di unghie, tutte le caratteristiche che ricorda genetica Epidermolisi bollosa distrofica. Questi anticorpi dell&#39;immunoglobulina G battesimo C7 sono patogeni, perch&eacute; quando essi vengono iniettati in topi, topi sviluppano una malattia vesciche EBA-come. Oltre alla presentazione di mechanobullous classica, EBA ha anche diversi altri distinte sindromi cliniche simili al pemfigoide bolloso, pemfigoide Brunsting Perry o Pemfigoide cicatriziale. Sebbene il trattamento per EBA &egrave; spesso insoddisfacente, qualche successo terapeutico &egrave; stato raggiunto con Colchicina, dapsone, plasmaferesi, fotoferesi, infliximab e immunoglobuline per via endovenosa.

Rishu Gupta

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