What is the history of Pemphigoid?

TL;DR: Pemphigoid was historically distinguished from pemphigus vulgaris in the mid-20th century, marking a major milestone in dermatological science. Today, it is recognized as a distinct autoimmune blistering disease caused by autoantibodies attacking the dermal-epidermal junction, a discovery that revolutionized its diagnosis and targeted treatment.

When was Pemphigoid first described and distinguished?

For centuries, the term "pemphigus" was used as a catch-all for any blistering skin condition. It was not until the 1950s that the medical community began to realize that not all blistering diseases were the same. In 1953, the physician Walter Lever performed a pivotal study that separated bullous pemphigoid from pemphigus vulgaris. By utilizing histopathology, Lever demonstrated that the blisters in pemphigoid formed beneath the epidermis (subepidermal), whereas those in pemphigus formed within the epidermis (intraepidermal). This distinction was critical because the two conditions required drastically different clinical approaches.

How has our understanding of Pemphigoid evolved?

The evolution of our understanding of pemphigoid has been driven by advancements in immunology. In the 1960s and 70s, researchers utilized immunofluorescence techniques to identify that pemphigoid is an autoimmune disorder. We now know that the body’s immune system produces autoantibodies that target specific proteins—specifically BP180 and BP230—located at the hemidesmosomes, which act as the "glue" anchoring the skin layers together. This shift from viewing it as a mysterious skin eruption to an antibody-mediated autoimmune disease allowed for the development of more precise diagnostic tests and therapies.

What are the major milestones in treatment and research?

The treatment landscape for pemphigoid has moved from non-specific immunosuppression to targeted biological therapies. Key milestones include:

• 1950s: The introduction of systemic corticosteroids, which became the standard of care for decades.


• 1980s-90s: The adoption of steroid-sparing agents (such as azathioprine or mycophenolate mofetil) to reduce the long-term side effects of high-dose steroids.


• 2000s-Present: The emergence of rituximab and other biologic agents that specifically deplete the B-cells responsible for producing the offending antibodies.

How did researchers correct historical misconceptions?

Historically, patients were often misdiagnosed because clinicians failed to biopsy the skin correctly or lacked the tools to identify the specific site of the blister. Early treatments often focused solely on the symptoms rather than the underlying mechanism. The correction came through the development of direct and indirect immunofluorescence, which allowed dermatologists to "see" the deposits of antibodies under a microscope. With 95 members on DiseaseMaps.org currently sharing their experiences, we see that modern patients benefit from this historical transition, as they are now able to receive faster, more accurate diagnoses compared to patients in the early 20th century.

Next steps

• Consult a board-certified dermatologist or a specialist in autoimmune blistering diseases for a skin biopsy and immunofluorescence testing.


• Connect with the 95 members of the pemphigoid community on DiseaseMaps.org to share lived experiences and coping strategies.


• Ask your physician about recent clinical trials involving targeted biologic therapies that may offer better outcomes than traditional high-dose steroids.


• Maintain a symptom diary to help your care team monitor the effectiveness of your current treatment plan.

Medical disclaimer: This content is for informational purposes only and does not constitute medical advice, diagnosis, or treatment; always seek the advice of your physician or other qualified health provider with any questions regarding a medical condition.

References

• NIH GARD: Bullous Pemphigoid Information Page.


• Orphanet: Rare disease database entry for Bullous Pemphigoid.


• PubMed: Historical review of the classification of blistering diseases (Lever, W.F., 1953).


• International Pemphigus & Pemphigoid Foundation (IPPF): Patient advocacy and clinical resources.