What are the symptoms? PV causes the skin to separate easily and peel easily. For many individuals, PV usually begins with blistering in the mouth and throat. This may be followed by blistering or erosions of the skin, including the groin, underarm, face, scalp and chest areas. PV lesions may cover extensive portions of the body. In some affected people the lesions are relatively asymptomatic. However, in the healing stage following treatment, the lesions often crust over but they no longer itch or burn and leak fluid. In some people the skin lesions may itch and burn continuously and rupture which may leave red erosions of the skin surrounded by a crust and scaling. Affected areas usually heal without scarring, unless the lesions become infected. Blisters in the mouth may make it difficult to eat and drink, leading to problems with weight loss and dehydration. What are the causes? PV is one of a group of chronic, relapsing conditions in which the immune system produces antibodies against specific proteins in the skin and mucous membrane, leading to the inability of the skin cells to bind together. It is thought that PV may be triggered by a range of factors. A few cases of PV have occurred following reactions to medications, including penicillamine and captopril. Individuals are probably genetically predisposed to PV. Specific information about a gene change(s) will not, however, indicate the certainty with which an individual will become affected with PV in the future. How is it diagnosed? Diagnosis of these diseases is made by skin biopsy (where a small amount of skin is removed for study in a laboratory) and immunopathology (analysis of body fluids to detect if there are any antibodies attacking the proteins in the skin and mucous membranes). How is it treated? There is no cure available for PV, but in most cases available treatments are highly successful in reducing symptoms and preventing complications. PV is often initially controlled with high-dose steroids together with one of a number of so-called ‘steroid sparing’ immunosuppressive drugs. Response to medication varies from individual to individual and, for some people, lesions may not heal for extended periods of time. The main risk from PV is infection and complications resulting from medication aimed at suppressing the immune system. Consequently doses are kept as low as possible, consistent with controlling symptoms. Inheritance patterns and prenatal diagnosis Inheritance patternsNone detectable but familial cases occur very rarely. Prenatal diagnosisNone available and not appropriate. Is there support? Pemphigus Vulgaris Network Helpline: 0208 863 3735Website: pemphigus.org.uk The Network is a support group for people living with Pemphigus and Mucous Membrane Pemphigoid. The helpline offers information and support to affected individuals, their families and carers, and medical professionals. Group details last updated October 2015.
What are the symptoms? PV causes the skin to separate easily and peel easily. For many individuals, PV usually begins with blistering in the mouth and throat. This may be followed by blistering or erosions of the skin, including the groin, underarm, face, scalp and chest areas. PV lesions may cover extensive portions of the body. In some affected people the lesions are relatively asymptomatic. However, in the healing stage following treatment, the lesions often crust over but they no longer itch or burn and leak fluid. In some people the skin lesions may itch and burn continuously and rupture which may leave red erosions of the skin surrounded by a crust and scaling. Affected areas usually heal without scarring, unless the lesions become infected. Blisters in the mouth may make it difficult to eat and drink, leading to problems with weight loss and dehydration.
What are the causes? PV is one of a group of chronic, relapsing conditions in which the immune system produces antibodies against specific proteins in the skin and mucous membrane, leading to the inability of the skin cells to bind together. It is thought that PV may be triggered by a range of factors. A few cases of PV have occurred following reactions to medications, including penicillamine and captopril. Individuals are probably genetically predisposed to PV. Specific information about a gene change(s) will not, however, indicate the certainty with which an individual will become affected with PV in the future.
How is it diagnosed? Diagnosis of these diseases is made by skin biopsy (where a small amount of skin is removed for study in a laboratory) and immunopathology (analysis of body fluids to detect if there are any antibodies attacking the proteins in the skin and mucous membranes).
How is it treated? There is no cure available for PV, but in most cases available treatments are highly successful in reducing symptoms and preventing complications. PV is often initially controlled with high-dose steroids together with one of a number of so-called ‘steroid sparing’ immunosuppressive drugs. Response to medication varies from individual to individual and, for some people, lesions may not heal for extended periods of time. The main risk from PV is infection and complications resulting from medication aimed at suppressing the immune system. Consequently doses are kept as low as possible, consistent with controlling symptoms.
Inheritance patterns and prenatal diagnosis Inheritance patternsNone detectable but familial cases occur very rarely. Prenatal diagnosisNone available and not appropriate.
Is there support? Pemphigus Vulgaris Network Helpline: 0208 863 3735Website: pemphigus.org.uk The Network is a support group for people living with Pemphigus and Mucous Membrane Pemphigoid. The helpline offers information and support to affected individuals, their families and carers, and medical professionals. Group details last updated October 2015.