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Autoimmune Bullous Diseases of Childhood

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Severe Skin Diseases in Children

Abstract

Autoimmune bullous diseases of childhood (ABDC) are a rare group of heterogeneous conditions that result from autoantibodies targeting antigens necessary for mucocutaneous integrity. Depending on the severity and extent of disease, systemic therapy may be necessary. Treatment is aimed at controlling blistering while avoiding adverse reactions. There are several anecdotal reports of treatment options, but controlled and comparative studies are lacking, and all therapies are off-label in children. This chapter will discuss the non-topical management options of ABDC in more detail, including dosage, adverse effects, and monitoring guidelines. The approaches to treatment of several specific subtypes of ABDC are then elaborated.

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Abbreviations

ABDC:

Autoimmune bullous diseases of childhood

ACE:

Angiotensin converting enzyme

BM:

Basement membrane

BP:

Bullous pemphigoid

CBDC:

Chronic bullous disease of childhood

DH:

Dermatitis herpetiformis

DRESS:

Drug reaction with eosinophilia and systemic symptoms

EBA:

Epidermolysis bullosa acquisita

ECP:

Extracorporeal photopheresis

G6PD:

Glucose-6-phosphate dehydrogenase

HPA:

Hypothalamic-pituitary-adrenal

Ig:

Immunoglobulin

INID:

Intraepidermal neutrophilic IgA dermatosis

IVIg:

Intravenous immune globulin

MMF:

Mycophenolate mofetil

MMP:

Mucous membrane pemphigoid

PCP:

Pneumocystis pneumonia

PF:

Pemphigus foliaceous

PPI:

Proton pump inhibitor

PV:

Pemphigus vulgaris

REMS:

Risk evaluation and mitigation strategy

SJS:

Stevens-Johnson syndrome

SLE:

Systemic lupus erythematosus

SPD:

Subcorneal pustular dermatosis

TEN:

Toxic epidermal necrolysis

TMP-SMX:

Trimethroprim-sulfamethoxazole

TPMT:

Thiopurine methyltransferase

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Authors and Affiliations

  1. College of Physicians and Surgeons, Columbia University College of Physicians and Surgeons, 630 West 168th Street, New York, NY, 10032, USA

    Nicole A. Weitz BA

  2. Department of Dermatology, Columbia University, 161 Fort Washington Avenue, 12th Floor, New York, NY, 10032, USA

    Emily M. Mintz MD & Kimberly D. Morel MD, FAAD, FAAP

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  1. Nicole A. Weitz BA
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  2. Emily M. Mintz MD
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  3. Kimberly D. Morel MD, FAAD, FAAP
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Editors and Affiliations

  1. Pediatric & Adolescent Dermatology, Rady Children's Hospital, San Diego, California, USA

    Wynnis L. Tom

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Weitz, N.A., Mintz, E.M., Morel, K.D. (2014). Autoimmune Bullous Diseases of Childhood. In: Tom, W. (eds) Severe Skin Diseases in Children. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-39532-1_5

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