Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy - 07/06/21

Doi : 10.1016/j.jaad.2020.05.161

Carmen M. Montagnon, MD ^a, Julia S. Lehman, MD ^a,^b, Dedee F. Murrell, MA, BMBCh, MD ^c, Michael J. Camilleri, MD ^a,^b, Stanislav N. Tolkachjov, MD ^d,^⁎
^a Department of Dermatology, Mayo Clinic, Rochester, Minnesota
^b Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota
^c Department of Dermatology, St George Hospital, University of New South Wales, Sydney, Australia
^d Epiphany Dermatology, Dallas, Texas

^∗Correspondence and reprint requests to: Stanislav N. Tolkachjov, MD, Epiphany Dermatology, 1640 FM 544, Suite 100, The Colony, TX 75056.Epiphany Dermatology1640 FM 544, Suite 100The ColonyTX75056

Abstract

Subepidermal (subepithelial) autoimmune blistering dermatoses are a group of rare skin disorders characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The fourth article in this continuing medical education series presents the current validated disease activity scoring systems, serologic parameters, treatments, and clinical trials for bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, bullous systemic lupus erythematosus, anti-p200 pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis.

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Key words : anti-p200 pemphigoid, autoimmune blistering disease severity index system, autoimmune bullous disease quality of life, bullous pemphigoid, bullous pemphigoid disease area index, bullous systemic lupus erythematosus, dapsone, dermatitis herpetiformis, epidermolysis bullosa acquisita, linear IgA bullous dermatosis, mucous membrane pemphigoid, subepithelial autoimmune bullous dermatoses

Abbreviations used : ABSIS, ABQOL, BP, BPDAI, DH, EBA, IVIG, MCID, MMP, PDAI, sCS, tCS