The Pemphigus & Pemphigoid Society

What is pemphigoid?

Pemphigoid is a group of rare autoimmune skin diseases that manifest themselves in burn-like lesions (blisters) that can cover a significant percentage of the skin and/or mucous membranes. Hospitalization may be required and it can be fatal if not properly treated. Pemphigoid is not contagious but it can be debilitating. Lesions are usually intense and painful. It is incurable but many patients go into long periods of remission. About half of pemphigoid patients have one or two flares before going into a long-time remission.

Generally pemphigoid is seen in people over 40 years of age. There are two main types of pemphigoid: bullous pemphigoid (BP) and cicatricial pemphigoid (CP). BP patients often suffer persistent and sometimes severe itch. Proper care of damaged skin is important to avoid infection and scarring. CP is more stubborn than BP and may affect the mucous membranes, the moist areas of skin including the mouth, throat and parts of the eye. Scarring is more frequent and this can cause serious problems such as blindness.

How is pemphigoid treated?

The drug of choice used to treat pemphigoid is a corticosteroid (steroid) such as prednisone or prednisolone and other medications that suppress the immune system. In very mild cases some patients require only topical steroids. Additional medications are required to treat some of the side effects of these medications. Withdrawal from these medications requires many months and must be done slowly under the supervision of a knowledgeable physician.

What is autoimmune disease? Autoimmune disease is caused by the immune system attacking the body's own organs or tissues. Autoimmune disease strikes one in every five Americans. The causes of autoimmune disease are unknown. When the immune system attacks the joints, it is called arthritis; when it attacks myelin (the insulation of the nerves) it is Multiple Sclerosis; when it attacks certain desmogleins (the “glue” that holds the skin together), it is a blistering disease called pemphigus.
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	> Essential information about pemphigoid What you need to know
	What is pemphigoid? Pemphigoid is a group of rare autoimmune skin diseases that manifest themselves in burn-like lesions (blisters) that can cover a significant percentage of the skin and/or mucous membranes. Hospitalization may be required and it can be fatal if not properly treated. Pemphigoid is not contagious but it can be debilitating. Lesions are usually intense and painful. It is incurable but many patients go into long periods of remission. About half of pemphigoid patients have one or two flares before going into a long-time remission. Generally pemphigoid is seen in people over 40 years of age. There are two main types of pemphigoid: bullous pemphigoid (BP) and cicatricial pemphigoid (CP). BP patients often suffer persistent and sometimes severe itch. Proper care of damaged skin is important to avoid infection and scarring. CP is more stubborn than BP and may affect the mucous membranes, the moist areas of skin including the mouth, throat and parts of the eye. Scarring is more frequent and this can cause serious problems such as blindness. How is pemphigoid treated? The drug of choice used to treat pemphigoid is a corticosteroid (steroid) such as prednisone or prednisolone and other medications that suppress the immune system. In very mild cases some patients require only topical steroids. Additional medications are required to treat some of the side effects of these medications. Withdrawal from these medications requires many months and must be done slowly under the supervision of a knowledgeable physician.	Prednisone is a very strong medication that can cause osteoporosis, diabetes, Cushing syndrome (moonface and humped back), depression, mental anxiety (possibly severe), mood swings, a runaway appetite, weight gain, an inability to concentrate, impaired judgment, physical weakness, muscle wasting and fatigue. Suppressing the immune system also makes the patient more susceptible to serious infection. Patients are advised to avoid anyone with a cold, flu or any infection and anyone recently exposed to any live virus such as polio or small pox vaccines. Patients using prednisone require adequate protein, low carbohydrate, low salt, low fat diet. Calcium with vitamin D supplements are advised. The prognosis for recovery is generally excellent, but it takes a long time to find the correct combination and amounts of medications to successfully control pemphigoid. An experienced doctor and supportive family are important factors in the degree and time of recovery.
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Essential information about pemphigoid

What you need to know