Pemphigus is a rare group of autoimmune diseases that causes blistering of the skin and the inside of the mouth, nose, throat, eyes, and genitals. With these diseases, the immune system mistakenly attacks healthy proteins that bind skin cells to one another, causing the skin to become fragile. Blisters then form from fluid that collects between the skin’s fragile layers.1,2
Though researchers do not know what causes the immune system to fight the body’s own proteins, they believe that both genetic and environmental factors are involved. Some people may be predisposed to the condition due to their genetic makeup, and something in their environment may trigger an immune response.
Signs and symptoms vary based on the type of pemphigus:1
Pemphigus vulgaris: The most common type in the US, blisters form within the mouth and other mucosal surfaces, as well as in a deep layer of the skin. They are often painful. Blisters can also form in the groin and under the arms in a subtype of the disease called pemphigus vegetans. Blisters typically start in the mouth and then appear on areas on the skin. The skin may become so fragile that it peels off by rubbing a finger on it.
Pemphigus foliaceus: Only affecting the skin, itchy or painful blisters form in upper skin layers. They typically appear first on the face, scalp, chest, or upper back, but can spread to other areas of skin on the body. The affected areas of skin may become inflamed and peel off in layers or scales.
Paraneoplastic pemphigus: Blisters or inflamed lesions typically form in the mouth and on the lips but may also develop on the skin and other mucosal surfaces. People with this type of pemphigus usually have a tumor and may have severe lung problems. If the tumor is surgically removed, the disease may improve.
IgA pemphigus: Caused by the IgA antibody, blisters or pimple-like bumps often form in groups or rings on the skin.
Drug-induced pemphigus: Pemphigus-like blisters or sores are triggered by certain medicines like antibiotics and blood pressure medications, as well as drugs that contain a chemical group called a thiol. When one stops taking the medication or drug, these blisters and sores typically go away.
Pemphigus is typically diagnosed through a skin biopsy, blood tests, and sometimes an endoscopy to check for sores in the throat.
Treatment is dependent on the type and severity of the disease. Medications like corticosteroid cream and prednisone pills are often prescribed as the first line of treatment intended to suppress blisters. To help suppress the immune system and keep it from attacking healthy proteins, medications such as azathioprine (Imuran, Azasan), mycophenolate (Cellcept) and cyclophosphamide are typically prescribed. If these treatment methods aren’t working, treatments such as intravenous immunoglobulin (IG) therapy are typically prescribed as a second or third-line treatment option.3
Intravenous immunoglobulin (IG) therapy is usually well tolerated with few rare serious side effects. It’s been used to treat dermatological conditions for more than two decades.4
How BioMatrix Can Help
BioMatrix has extensive experience with IG-related support services. As a national provider of IG, BioMatrix has broad access to all brands and inventory. Our IG treatment plans are designed to:
Prevent infections
Boost the immune system
Avoid complications of therapy
Prevent long-term organ damage
Decrease hospitalizations
Encourage patients to participate in disease management
Prolong life
Improve general health and quality of life
Our nursing team coordinates the best site of care for scheduled infusions—whether in the patient’s home or physician’s office. Providing site-of-care options offers convenience for patients and cost savings to insurance providers.
Together, our clinicians, support staff, and digital health technology offer a comprehensive approach improving quality of life for patients and producing positive outcomes along the entire healthcare continuum.
DISCLAIMER: THIS IS NOT MEDICAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.
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References
(2021). Pemphigus. National Institutes of Health. https://www.niams.nih.gov/health-topics/pemphigus
Pemphigus Vulgaris. John Hopkins Medicine. https://www.hopkinsmedicine.org/health/conditions-and-diseases/pemphigus-vulgaris
(2022). Pemphigus. Mayo Foundation for Medical Education and Research (MFMER). https://www.mayoclinic.org/diseases-conditions/pemphigus/diagnosis-treatment/drc-20350409
Hoffmann, J.H.O. and Enk, A.H. (2017), High-dose intravenous immunoglobulins for the treatment of dermatological autoimmune diseases. JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 15: 1211-1226. https://doi.org/10.1111/ddg.13389
