Overview
Anti-p200 pemphigoid is a rare autoimmune blistering skin disease. In this condition, the body's immune system mistakenly attacks a protein called laminin gamma-1 (also known as the p200 antigen), which is found at the junction between the outer layer of skin (epidermis) and the deeper layer (dermis). This attack causes the layers of skin to separate, leading to the formation of tense blisters and widespread itchy, red skin lesions. The disease is sometimes also called anti-laminin gamma-1 pemphigoid. Patients typically develop tense fluid-filled blisters on the skin that can appear on the trunk, arms, legs, and sometimes the hands and feet. The blisters may resemble those seen in bullous pemphigoid, but anti-p200 pemphigoid is distinguished by the specific antibody target. Some patients also develop lesions on mucous membranes, such as the mouth. Itching can be severe and significantly affect quality of life. Treatment usually involves medications that suppress the immune system to reduce blister formation. First-line therapy often includes systemic corticosteroids such as prednisone, sometimes combined with steroid-sparing agents like dapsone, mycophenolate mofetil, or azathioprine. Many patients respond well to treatment, though the disease can be chronic and may relapse. Because this condition is so rare, treatment approaches are largely based on case reports and small case series rather than large clinical trials.
Key symptoms:
Tense fluid-filled blisters on the skinIntense itchingRed or inflamed patches of skinBlisters on the hands and feetBlisters on the trunk, arms, and legsHive-like raised red patches (urticarial plaques)Mouth sores or blisters in some casesSkin erosions where blisters have broken openCrusting of skin lesionsScarring in some cases
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Anti-p200 pemphigoid.
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Specialists
View all specialists →No specialists are currently listed for Anti-p200 pemphigoid.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Anti-p200 pemphigoid.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.How was the diagnosis of anti-p200 pemphigoid confirmed, and what tests were done?,What treatment options do you recommend, and what are the potential side effects?,How long will I need to take medication, and can the disease go into remission?,What should I do if I develop new blisters or signs of a flare while on treatment?,Are there any activities or triggers I should avoid to prevent flare-ups?,How often will I need blood tests or follow-up visits to monitor my condition and medications?,Should I see any other specialists in addition to my dermatologist?
Common questions about Anti-p200 pemphigoid
What is Anti-p200 pemphigoid?
Anti-p200 pemphigoid is a rare autoimmune blistering skin disease. In this condition, the body's immune system mistakenly attacks a protein called laminin gamma-1 (also known as the p200 antigen), which is found at the junction between the outer layer of skin (epidermis) and the deeper layer (dermis). This attack causes the layers of skin to separate, leading to the formation of tense blisters and widespread itchy, red skin lesions. The disease is sometimes also called anti-laminin gamma-1 pemphigoid. Patients typically develop tense fluid-filled blisters on the skin that can appear on the tr
How is Anti-p200 pemphigoid inherited?
Anti-p200 pemphigoid follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Anti-p200 pemphigoid typically begin?
Typical onset of Anti-p200 pemphigoid is adult. Age of onset can vary across affected individuals.