A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita.

Abstract

Autoimmune blistering disease management can be challenging as treatment modalities vary greatly and no single standard of care exists. We consolidated the recommendations of international management guidelines in order to provide optimal management suggestions to physicians. A comprehensive literature search in PubMed/MEDLINE for published blistering disease management guidelines and consensus statements was conducted in November 2019. Search terms included "guideline or guidelines" or "consensus" and "pemphigoid" or "autoimmune blistering disease" or "epidermolysis bullosa acquisita". We included guidelines from established dermatologic societies and expert consensus groups. We excluded literature reviews, guidelines established by an association without dermatologists, or those specific to a single treatment. Guidelines in all languages were considered. Eleven guidelines from dermatologic associations and consensus groups meeting our inclusion criteria were selected. Several differences between recommendations, most notably when to introduce adjuvants for refractory disease, were found in bullous pemphigoid. In mucous membrane pemphigoid, treatment was directed to the sites involved and managed with systemic corticosteroids and immunosuppressants/biologics. There was no universal consensus on the first-line treatment for epidermolysis bullosa acquisita, but a combination of immunosuppressive, anti-inflammatory, and anti-neutrophil therapy was utilized. Comparison of the management guidelines revealed underrepresentation of guidelines from developing nations and key differences between the management styles among dermatologists from Europe and Asia. We attribute these discrepancies to the time elapsed between guidelines, regional differences, and demands of the local healthcare systems.

• Authors: Payal M Patel, Virginia A Jones, Taryn N Murray, Kyle T Amber
• Journal: American journal of clinical dermatology (Am J Clin Dermatol) Vol. 21 Issue 4 Pg. 557-565 (Aug 2020) ISSN: 1179-1888 [Electronic] New Zealand
• PMID: 32180161 (Publication Type: Comparative Study, Journal Article, Review)

Chemical References

• Anti-Inflammatory Agents
• Biological Products
• Dermatologic Agents
• Immunosuppressive Agents

Topics

• Administration, Oral
• Administration, Topical
• Anti-Inflammatory Agents (administration & dosage, adverse effects)
• Biological Products (administration & dosage, adverse effects)
• Consensus
• Dermatologic Agents (administration & dosage)
• Dermatology (methods, standards)
• Drug Resistance
• Epidermolysis Bullosa Acquisita (diagnosis, drug therapy, immunology)
• Female
• Humans
• Immunosuppressive Agents (administration & dosage, adverse effects)
• Pemphigoid Gestationis (diagnosis, drug therapy, immunology)
• Pemphigoid, Benign Mucous Membrane (diagnosis, drug therapy, immunology)
• Pemphigoid, Bullous (diagnosis, drug therapy, immunology)
• Practice Guidelines as Topic
• Pregnancy
• Severity of Illness Index
• Treatment Outcome