Facts about Pemphigus foliaceus
PEMPHIGUS is one of a group of rare, relapsing auto-immune diseases, causing blistering of the skin and, for the type known as pemphigus vulgaris, mucous membranes like mouth, nose, throat and genitals. It occurs in both humans and animals.
PEMPHIGUS is one of a group of rare, relapsing auto-immune diseases, causing blistering of the skin and, for the type known as pemphigus vulgaris, mucous membranes like mouth, nose, throat and genitals. It occurs in both humans and animals.
What happens?
The immune system views cells in the skin and mucous membranes as foreign bodies and attacks them. The cells no longer stick together and this causes the burn-like lesions or blisters.
Pemphigus foliaceus is found in the top layer of the skin, so mouth sores do not occur. The patient is usually in good health. Small fluid-filled blisters first form on the skin surface, rupture very easily. It is characterised by crusty sores that often begin on the scalp, and may move to the chest, back and face. A burning sensation or localised pain may be felt. It is often mis-diagnosed as dermatitis or eczema.
What causes it?
It is not known why people develop these auto-antibodies. Experts point to genetic factors, but there are other factors that trigger it off, including use of pesticides, malignancy, reaction to drugs, errant hormones, infectious agents, food allergies, ultraviolet rays and stress.
How common is it?
It is very rare and sporadic worldwide. Mulago Hospital gets two to three patients a year. It is common among 50-60-year-old, but can occur at any age in children, young adults, and genetically-related family members.
How is it treated?
There is no specific cure available, but the condition is controllable with heavy immunosuppressive treatment. But if left untreated, pemphigus is fatal due to overwhelming systemic infection.
The primary aim of treatment is to prevent new areas from developing infections and promote healing of affected areas.
Topical treatment with corticosteroids and antibiotics is usually all that is necessary for mild cases. The most common treatment is the administration of oral steroids, especially prednisone.
All drugs may cause severe side effects because you have to begin with very high doses, so the patient must be closely monitored by doctors. Once the outbreaks are under control, dosage is often reduced, to lessen side effects.
Sometimes treatment heals it completely so that it does not flare-up when treatment is stopped. This is called being in complete remission.
Sadly, some people never get complete remission and have to stay on relatively small doses of their medication to keep it under control. The condition may relapse, which means that patients should expect to have flare-ups after it gets better. Each flare-up can be worse or better than the last one.
There is no way of knowing when these flare-ups might happen or how bad they could be but they most commonly occur in the first two years following treatment.
Herbal treatment
Experts warn that herbal medicine should not be used because it usually increases the activity of the immune system. Yet, in order to control pemphigus foliaceus, it is currently thought necessary to suppress activity — the direct opposite.
Usually, a herbal practitioner may not even be able to tell you whether the medicine they are prescribing increases immune activity or not.
Compiled by Jackie Nake
What happens?
The immune system views cells in the skin and mucous membranes as foreign bodies and attacks them. The cells no longer stick together and this causes the burn-like lesions or blisters.
Pemphigus foliaceus is found in the top layer of the skin, so mouth sores do not occur. The patient is usually in good health. Small fluid-filled blisters first form on the skin surface, rupture very easily. It is characterised by crusty sores that often begin on the scalp, and may move to the chest, back and face. A burning sensation or localised pain may be felt. It is often mis-diagnosed as dermatitis or eczema.
What causes it?
It is not known why people develop these auto-antibodies. Experts point to genetic factors, but there are other factors that trigger it off, including use of pesticides, malignancy, reaction to drugs, errant hormones, infectious agents, food allergies, ultraviolet rays and stress.
How common is it?
It is very rare and sporadic worldwide. Mulago Hospital gets two to three patients a year. It is common among 50-60-year-old, but can occur at any age in children, young adults, and genetically-related family members.
How is it treated?
There is no specific cure available, but the condition is controllable with heavy immunosuppressive treatment. But if left untreated, pemphigus is fatal due to overwhelming systemic infection.
The primary aim of treatment is to prevent new areas from developing infections and promote healing of affected areas.
Topical treatment with corticosteroids and antibiotics is usually all that is necessary for mild cases. The most common treatment is the administration of oral steroids, especially prednisone.
All drugs may cause severe side effects because you have to begin with very high doses, so the patient must be closely monitored by doctors. Once the outbreaks are under control, dosage is often reduced, to lessen side effects.
Sometimes treatment heals it completely so that it does not flare-up when treatment is stopped. This is called being in complete remission.
Sadly, some people never get complete remission and have to stay on relatively small doses of their medication to keep it under control. The condition may relapse, which means that patients should expect to have flare-ups after it gets better. Each flare-up can be worse or better than the last one.
There is no way of knowing when these flare-ups might happen or how bad they could be but they most commonly occur in the first two years following treatment.
Herbal treatment
Experts warn that herbal medicine should not be used because it usually increases the activity of the immune system. Yet, in order to control pemphigus foliaceus, it is currently thought necessary to suppress activity — the direct opposite.
Usually, a herbal practitioner may not even be able to tell you whether the medicine they are prescribing increases immune activity or not.
Compiled by Jackie Nake