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Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management.
Clin Rev Allergy Immunol. 2018 Feb; 54(1):26-51.CR

Abstract

Autoimmune subepidermal blistering diseases of the skin and mucosae constitute a large group of sometimes devastating diseases, encompassing bullous pemphigoid, gestational pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. Their clinical presentation is polymorphic. These autoimmune blistering diseases are associated with autoantibodies that target distinct components of the basement membrane zone of stratified epithelia. These autoantigens represent structural proteins important for maintenance of dermo-epidermal integrity. Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease of the skin and mucosae. Although the disease typically presents with a generalized blistering eruption associated with itch, atypical variants with either localized bullous lesions or "non-bullous" presentations are observed in approximately 20% of patients. A peculiar form of BP typically associated with pregnancy is pemphigoid gestationis. In anti-p200 pemphigoid, patients present with tense blisters on erythematosus or normal skin resembling BP, with a predilection for acral surfaces. These patients have antibodies targeting the 200-kDa basement membrane protein. Epidermolysis bullosa is a rare autoimmune blistering disease associated with autoantibodies against type VII collagen that can have several phenotypes including a classical form mimicking dystrophic epidermolysis bullosa, an inflammatory presentation mimicking BP, or mucous membrane pemphigoid-like lesions. Mucous membrane pemphigoid (MMP) is the term agreed upon by international consensus for an autoimmune blistering disorder, which affects one or more mucous membrane and may involve the skin. The condition involves a number of different autoantigens in the basement membrane zone. It may result in severe complications from scarring, such as blindness and strictures. Diagnosis of these diseases relies on direct immunofluorescence microscopy studies and immunoserological assays. Management of affected patients is often challenging. We will here review the clinical and immunopathological features as well as the pathophysiology of this group of organ-specific autoimmune diseases. Finally, we will discuss the diagnostic approach and the principles of management in clinical practice.

Authors+Show Affiliations

Department of Dermatology, University of California Irvine Health, 118 Med Surg 1, Irvine, CA, 92697, USA. KAmber@UCI.edu.Department of Dermatology, St. George Hospital, Gray Street, Kogarah, Sydney, NSW, Australia.Department of Dermatology, University of Lübeck, Lübeck, Germany. Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany.Department of Dermatology, INSERM U901, University of Rouen, Rouen, France.Department of Dermatology, University of Bern, Bern, Switzerland.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

28779299

Citation

Amber, Kyle T., et al. "Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management." Clinical Reviews in Allergy & Immunology, vol. 54, no. 1, 2018, pp. 26-51.
Amber KT, Murrell DF, Schmidt E, et al. Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management. Clin Rev Allergy Immunol. 2018;54(1):26-51.
Amber, K. T., Murrell, D. F., Schmidt, E., Joly, P., & Borradori, L. (2018). Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management. Clinical Reviews in Allergy & Immunology, 54(1), 26-51. https://doi.org/10.1007/s12016-017-8633-4
Amber KT, et al. Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management. Clin Rev Allergy Immunol. 2018;54(1):26-51. PubMed PMID: 28779299.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management. AU - Amber,Kyle T, AU - Murrell,Dedee F, AU - Schmidt,Enno, AU - Joly,Pascal, AU - Borradori,Luca, PY - 2017/8/6/pubmed PY - 2018/8/17/medline PY - 2017/8/6/entrez KW - Anti-p200 pemphigoid KW - Autoimmune blistering disease KW - Bullous pemphigoid KW - Epidermolysis bullosa acquisita KW - Pemphigoid gestationis SP - 26 EP - 51 JF - Clinical reviews in allergy & immunology JO - Clin Rev Allergy Immunol VL - 54 IS - 1 N2 - Autoimmune subepidermal blistering diseases of the skin and mucosae constitute a large group of sometimes devastating diseases, encompassing bullous pemphigoid, gestational pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. Their clinical presentation is polymorphic. These autoimmune blistering diseases are associated with autoantibodies that target distinct components of the basement membrane zone of stratified epithelia. These autoantigens represent structural proteins important for maintenance of dermo-epidermal integrity. Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease of the skin and mucosae. Although the disease typically presents with a generalized blistering eruption associated with itch, atypical variants with either localized bullous lesions or "non-bullous" presentations are observed in approximately 20% of patients. A peculiar form of BP typically associated with pregnancy is pemphigoid gestationis. In anti-p200 pemphigoid, patients present with tense blisters on erythematosus or normal skin resembling BP, with a predilection for acral surfaces. These patients have antibodies targeting the 200-kDa basement membrane protein. Epidermolysis bullosa is a rare autoimmune blistering disease associated with autoantibodies against type VII collagen that can have several phenotypes including a classical form mimicking dystrophic epidermolysis bullosa, an inflammatory presentation mimicking BP, or mucous membrane pemphigoid-like lesions. Mucous membrane pemphigoid (MMP) is the term agreed upon by international consensus for an autoimmune blistering disorder, which affects one or more mucous membrane and may involve the skin. The condition involves a number of different autoantigens in the basement membrane zone. It may result in severe complications from scarring, such as blindness and strictures. Diagnosis of these diseases relies on direct immunofluorescence microscopy studies and immunoserological assays. Management of affected patients is often challenging. We will here review the clinical and immunopathological features as well as the pathophysiology of this group of organ-specific autoimmune diseases. Finally, we will discuss the diagnostic approach and the principles of management in clinical practice. SN - 1559-0267 UR - https://www.unboundmedicine.com/medline/citation/28779299/Autoimmune_Subepidermal_Bullous_Diseases_of_the_Skin_and_Mucosae:_Clinical_Features_Diagnosis_and_Management_ L2 - https://doi.org/10.1007/s12016-017-8633-4 DB - PRIME DP - Unbound Medicine ER -