Intraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis - 07/05/21

Doi : 10.1016/j.jaad.2020.11.075

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

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

Abstract

Intraepithelial autoimmune blistering dermatoses are a rare group of skin disorders characterized by the intraepithelial disruption of intercellular connections through the action of autoantibodies. The first article in this continuing medical education series explores the background, epidemiology, clinical features, and diagnostic criteria of each of the major intraepithelial autoimmune blistering dermatoses, including pemphigus foliaceus, pemphigus erythematosus, pemphigus herpetiformis, fogo selvagem, pemphigus vulgaris, pemphigus vegetans, drug-induced pemphigus, IgA pemphigus, IgG/IgA pemphigus, and paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome.

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Key words : drug-induced pemphigus, fogo selvagem, IgA pemphigus, IgA/IgG pemphigus, paraneoplastic autoimmune multiorgan syndrome, paraneoplastic pemphigus, pemphigus erythematosus, pemphigus foliaceus, pemphigus herpetiformis, pemphigus vegetans, pemphigus vulgaris

Abbreviations used : DIF, DSG, ELISA, FS, HLA, IIF, PAMS, PE, PF, PH, PNP, PV, PVe, SPD

Plan

Introduction

Superficial IgG pemphigus: pemphigus foliaceus

Key points

Clinical features

Diagnosis

Superficial IgG pemphigus: pemphigus erythematosus

Key point

Clinical features

Diagnosis

Superficial IgG pemphigus: fogo selvagem

Suprabasilar IgG pemphigus: pemphigus vulgaris

Background

Clinical features

Diagnosis

Suprabasilar IgG pemphigus: pemphigus vegetans

Key point

Clinical features

Diagnosis

Other forms of IgG pemphigus: pemphigus herpetiformis

Key point

Clinical features

Diagnosis

Other forms of IgG pemphigus: drug-induced pemphigus

Key point

Clinical features

Diagnosis

Other forms of IgG pemphigus: paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome

Other forms of pemphigus: IgA pemphigus

Other forms of pemphigus: IgG/IgA pemphigus

	Funding sources: None.
	Date of release: June 2021.
	Expiration date: June 2024.
	IRB approval status: Not applicable.
	Reprints not available from the authors.