Endemic Pemphigus Foliaceus (EPF)

Last updated: February 8, 2026Reviewed date: February 8, 2026Reading time: 8 min read
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Article Summary

Endemic pemphigus foliaceus (EPF) is a rare autoimmune skin disease characterized by chronic and recurrent eruptions of blistering, scaling and crusting skin lesions. It is also known as fogo selvagem or wildfire. EPF is endemic or restricted to specific geographic areas, typically in rural regions of South America, Africa, and the Middle East. The disease affects individuals of all ages and is more common in...

Key Takeaways

  • This article explains Causes in simple medical language.
  • This article explains Symptoms in simple medical language.
  • This article explains Diagnosis in simple medical language.
  • This article explains Treatment in simple medical language.
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Definition

Endemic pemphigus foliaceus (EPF) is a rare skin disease characterized by and eruptions of blistering, scaling and crusting skin lesions. It is also known as fogo selvagem or wildfire. EPF is endemic or restricted to specific geographic areas, typically in rural regions of South America, Africa, and the Middle East. The disease affects individuals of all ages and is more common in males. The exact cause of EPF is unknown, but it is believed to be triggered by environmental factors such as insect bites, infections, or exposure to certain chemicals. The disease is characterized by antibodies produced by the immune system that attack and destroy the skin cells causing the formation of blisters and skin lesions.

Causes

The exact cause of EPF is unknown, but several factors are believed to contribute to its development, including:

  1. Genetics: EPF is more common in certain populations and is believed to have a component.
  2. Environmental factors: EPF is found in specific regions of the world, and exposure to certain environmental factors such as sunlight, temperature, and humidity may contribute to its development.
  3. Infections: Some studies suggest that infections may trigger the development of EPF, but the exact mechanism is unclear.
  4. Immune system dysfunction: EPF is an , meaning that the immune system attacks healthy skin cells. This may be due to an overactive immune response, or a failure of the immune system to recognize and tolerate normal skin cells.
  5. Stress: Chronic stress may contribute to the development of autoimmune diseases, including EPF.

Overall, the causes of EPF are complex and , and the exact mechanisms remain unclear. Further research is needed to better understand the underlying causes of this disease.

Symptoms

Endemic pemphigus foliaceus is a type of autoimmune skin disease that affects people living in specific regions of the world, particularly in South and Central America. The main symptoms of this condition include:

  1. Rashes and Blisters: A or blisters may develop on the face, neck, scalp, and upper trunk. These blisters are filled with fluid and may break open, leading to raw or painful skin.
  2. Scaling and Crusting: The affected skin may become dry and scaly, and crusting may occur around the blisters.
  3. : The skin may be itchy, especially in areas where the blisters are located.
  4. Eyelid : Swelling of the eyelids may occur, causing vision problems and discomfort.
  5. Hair Loss: Hair loss may occur in areas affected by the blisters and rashes, especially on the scalp.
  6. Nail Changes: The nails may become thick, brittle, and discolored, and the skin around the nails may become red and painful.
  7. : People with endemic pemphigus foliaceus may feel tired and have a general feeling of being unwell.

It is important to note that the symptoms of this condition may vary from person to person and may develop slowly over time. If you experience any of these symptoms, it is important to seek medical attention as soon as possible.

The diagnosis of EPF is typically based on examination, laboratory tests, and histopathologic examination of skin biopsies.

Clinical examination: The clinical presentation of EPF is characterized by the presence of multiple crusted and/or non-crusted erosions and blisters on the face, neck, trunk, and extremities. The lesions may be widespread and progressive.

Laboratory tests: Blood tests are performed to help confirm the diagnosis of EPF. Antinuclear antibodies (ANA) and anti-dsDNA antibodies are often positive in EPF patients. Serum levels of immunoglobulins (IgG, IgM, and IgA) may also be elevated.

Histopathologic examination: A skin is performed to examine the underlying skin tissue for characteristic features of EPF. This includes the presence of acantholysis (the separation of cells in the top layer of the skin), a subcorneal , and infiltration of lymphocytes and eosinophils in the .

  1. Skin biopsy: A small sample of the affected skin is taken for examination under a microscope. This can help confirm the presence of EPF and rule out other skin conditions.
  2. Direct immunofluorescence: This test involves applying a special dye to a sample of the affected skin and then examining it under a microscope. The presence of antibodies in the skin can be seen using this test.
  3. Indirect immunofluorescence: This test involves taking a sample of the patient’s blood and examining it under a microscope. The presence of antibodies in the blood can indicate the presence of EPF.
  4. ELISA test: This test involves measuring the levels of antibodies in the blood using a blood sample. Elevated levels of antibodies can indicate the presence of EPF.
  5. PCR test: This test involves taking a sample of the affected skin and examining it for the presence of specific genes that are associated with EPF.

In summary, the diagnosis of EPF is based on a combination of clinical examination, laboratory tests, and histopathologic examination of skin biopsies. Confirming the diagnosis is important for proper treatment and management of the condition.

Treatment

Endemic pemphigus foliaceus is a rare autoimmune skin disease that affects people living in certain regions of the world. The main treatment for this condition involves a combination of medications and topical treatments.

  1. Corticosteroids: Corticosteroids are powerful drugs that are commonly used to treat autoimmune diseases. They help to suppress the immune system and reduce in the skin.
  2. Immunosuppressive Drugs: Drugs such as cyclophosphamide, azathioprine, and methotrexate can also be used to suppress the immune system. They are often used in combination with corticosteroids to increase their effectiveness.
  3. Rituximab: This is a monoclonal antibody that targets the B-cells in the immune system. By targeting these cells, it helps to reduce the severity of the autoimmune response.
  4. Topical Treatments: Topical treatments such as corticosteroid creams, ointments, and lotions can be used to treat skin lesions. They can be used in conjunction with systemic treatments to control the symptoms of the disease.
  5. Antihistamines: Antihistamines can be used to relieve itching, which is a common symptom of endemic pemphigus foliaceus.
  6. Phototherapy: Phototherapy, or light therapy, can also be used to treat skin lesions. This involves exposing the skin to ultraviolet light, which can help to reduce inflammation and improve the appearance of the skin.

Topical corticosteroids are usually the first line of treatment and are applied directly to the affected skin areas. Systemic corticosteroids, such as prednisone, may also be used for more cases.

In addition to corticosteroids, other immunosuppressive medications may be used to treat pemphigus foliaceus, such as cyclophosphamide or azathioprine. These medications work by suppressing the immune system, which can reduce the production of antibodies that attack the skin.

Plasmapheresis and intravenous immunoglobulin (IVIg) may also be used as treatments for pemphigus foliaceus. Plasmapheresis involves removing the patient’s plasma and replacing it with fresh plasma, which can help to reduce the amount of antibodies in the blood. IVIg is a treatment that involves injecting high doses of immunoglobulins into the patient, which can help to suppress the immune system.

In severe cases of pemphigus foliaceus, treatment may also include antibiotics to prevent skin infections. In addition, patients may be advised to avoid sunlight and use sunscreens, as sun exposure can worsen symptoms.

Overall, the main goal of treatment for endemic pemphigus foliaceus is to reduce inflammation, suppress the immune system, and prevent skin infections. Treatment may require a combination of medications, topical treatments, and lifestyle changes to achieve the best results.

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A simple rural-patient checklist to help you explain symptoms clearly, ask better questions, and avoid unsafe self-treatment.

Safety note: This is not a prescription or diagnosis. For severe symptoms, pregnancy danger signs, children with serious illness, chest pain, breathing difficulty, stroke-like weakness, or major injury, seek urgent care.

Which doctor may help?

Start with a registered doctor or the nearest qualified health center.

What to tell the doctor

  • Write when the problem started and how it changed.
  • Bring old prescriptions, investigation reports, and current medicines.
  • Write allergies, pregnancy status, diabetes, kidney/liver disease, and major past illnesses.
  • Bring one family member if the patient is weak, elderly, confused, or a child.

Questions to ask

  • What is the most likely cause of my symptoms?
  • Which danger signs mean I should go to hospital quickly?
  • Which tests are necessary now, and which can wait?
  • How should I take medicines safely and what side effects should I watch for?
  • When should I come for follow-up?

Tests to discuss

  • Vital signs: temperature, pulse, blood pressure, oxygen saturation
  • Basic physical examination by a clinician
  • CBC, urine test, blood sugar, or imaging only when clinically needed

Avoid these mistakes

  • Do not use antibiotics, steroid tablets/injections, or strong painkillers without proper medical advice.
  • Do not hide pregnancy, kidney disease, ulcer, allergy, or blood thinner use.
  • Do not delay emergency care when danger signs are present.

Medicine safety and first-aid guide

This section is for patient education only. It does not replace a doctor, pharmacist, or emergency care.

Safe first steps

  • Avoid heavy lifting, sudden bending, and prolonged bed rest.
  • Use comfortable posture and gentle movement as tolerated.
  • Discuss physiotherapy, X-ray, or MRI only when clinically needed.

OTC medicine safety

  • For mild back pain, pain-relief medicine may be discussed with a doctor or pharmacist.
  • Avoid repeated painkiller use if you have kidney disease, stomach ulcer, uncontrolled blood pressure, or are taking blood thinners.

Avoid these mistakes

  • Do not start antibiotics without a proper medical decision.
  • Do not use steroid tablets or injections casually for quick relief.
  • Do not delay emergency care because of home remedies.

Get urgent help if

  • Back pain with leg weakness, numbness around private area, loss of urine/stool control, fever, cancer history, or major injury needs urgent care.
Medicine names, dose, and timing must be decided by a qualified clinician or pharmacist after checking age, pregnancy, allergy, other diseases, and current medicines.

For rural patients and family caregivers

Patient health record and symptom diary

Write your symptoms, medicines already taken, test results, and questions before visiting a doctor. This note stays on your device unless you print or copy it.

Doctor to discuss: Doctor / qualified healthcare provider
Tests to discuss with doctor
  • Basic vital signs: temperature, pulse, blood pressure, oxygen level if needed
  • Relevant blood, urine, imaging, or specialist tests only after clinical assessment
Questions to ask
  • What is the most likely cause of my symptoms?
  • Which warning signs mean I should go to emergency care?
  • Which tests are really needed now?
  • Which medicines are safe for my age, pregnancy status, allergy, kidney/liver/stomach condition, and current medicines?

Emergency warning signs such as chest pain, severe breathing difficulty, sudden weakness, confusion, severe dehydration, major injury, or loss of bladder/bowel control need urgent medical care. Do not wait for online information.

Safe pathway to proper treatment

Care roadmap for: Endemic Pemphigus Foliaceus (EPF)

Use this simple roadmap to understand the next safe steps. It is educational and does not replace examination by a doctor.

Go to emergency care if you notice:
  • Severe or rapidly worsening symptoms
  • Breathing difficulty, chest pain, fainting, confusion, severe weakness, major injury, or severe dehydration
Doctor / service to discuss: Qualified healthcare provider; specialist depends on symptoms and examination.
  1. Step 1

    Check danger signs first

    If danger signs are present, seek emergency care and do not wait for online information.

  2. Step 2

    Record the symptom story

    Write when symptoms started, severity, medicines already taken, allergies, pregnancy status, and test results.

  3. Step 3

    Visit a qualified clinician

    A doctor, nurse, or qualified healthcare provider can examine you and decide which tests or treatment are needed.

  4. Step 4

    Do only useful tests

    Do tests after clinical assessment. Avoid unnecessary tests, random antibiotics, or repeated medicines without diagnosis.

  5. Step 5

    Follow up and return early if worse

    If symptoms worsen, new warning signs appear, or treatment is not helping, return for review quickly.

Rural patient practical tips
  • Take a written symptom diary and all previous prescriptions/test reports.
  • Do not hide medicines already taken, even herbal or over-the-counter medicines.
  • Ask which warning signs mean urgent referral to hospital.

This roadmap is for education. A real diagnosis and treatment plan requires history, examination, and clinical judgment.