What are the other Names for this Condition? (Also known as/Synonyms)

• Amendola Syndrome
• Endemic Pemphigus Foliaceus
• Wildfire Pemphigus

What is Fogo Selvagem? (Definition/Background Information)

• Fogo Selvagem is a rare variant of pemphigus foliaceus, a skin disorder, that is uniquely endemic to certain regions of rural Brazil and surrounding nations. It is also known as Endemic Pemphigus Foliaceus, and mostly affects younger individuals
• Pemphigus family of skin diseases are serious acute and chronic skin disorders commonly affecting mid-to-elderly adults. It is characterized by the formation of painful blisters on the surface of the skin and mucous membranes, and is caused by immune system dysregulation
• Fogo Selvagem is characterized by superficial blisters that develop over many weeks on the face, neck, and upper torso, which present severe burning sensation. Untreated cases may result in severe complications such as infections and growth delays in children
• The treatment of choice for Fogo Selvagem is topical and oral corticosteroids. With prompt and appropriate therapy, the outcomes are usually good, and no long-term complications are noted

Who gets Fogo Selvagem? (Age and Sex Distribution)

• Fogo Selvagem is generally observed in children and young adults, but individuals of other age groups may also be affected
• Both males and females are affected, and no gender predilection is seen

The skin disorder is specifically observed in certain parts of South America. The endemic regions include south and Midwest Brazil, Colombia, El Salvador, Paraguay, and Peru. A few cases have also been reported from the African nation of Tunisia.

What are the Risk Factors for Fogo Selvagem? (Predisposing Factors)

The risk factors for Fogo Selvagem include:

• People living in the endemic (particularly rural) regions of Brazil and other surrounding countries
• Living near waterbodies such as rivers and streams
• Low socioeconomic status
• Malnutrition
• Genetic susceptibility due to association with HLA complex
• Black fly bites are believed to trigger the condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Fogo Selvagem? (Etiology)

The exact cause of development of Fogo Selvagem is not well-understood. Research experts inform that it may develop due to a combination of genetic and environmental factors.

Fogo Selvagem is described as an autoimmune (blistering) disorder of skin in which an individual's immune system attacks their own skin components, due to the production of immunoglobulin antibodies.

• This results in the characteristic signs and symptoms of the disorder
• It is thought that the bite of the black fly, Simulium nigrimanum, may trigger this autoimmune condition
• Fogo Selvagem is not a contagious skin condition. It cannot be transmitted from one individual to another through direct physical contact or sharing of materials

What are the Signs and Symptoms of Fogo Selvagem?

The signs and symptoms of Fogo Selvagem may include:

• Presence of fluid-filled blisters on skin; the blisters/vesicles involve the superficial skin layers
• The blisters may rupture when rubbed, called the Nikolsky's sign
• A characteristic burning sensation is felt on skin, which gives the disorder its name ‘Fogo Selvagem’, meaning “wildfire”
• The condition may progress over many weeks and months, affecting the face (including the scalp), neck, chest, and upper back areas
• The lesions erode and result in crust formation
• The mucosal surfaces are generally not affected

How is Fogo Selvagem Diagnosed?

A diagnosis of Fogo Selvagem may involve the following exams and procedures:

• Physical examination of the individual and medical history evaluation
• Nikolsky sign: The physician may rub the skin near the blister to check if the skin peels-off. If it does, it is an indication of pemphigus. The Nikolsky sign is also seen in toxic epidermal necrolysis, Stevens-Johnson syndrome, and staphylococcal scalded skin syndrome
• Blood tests: Blood tests are conducted to check the level of desmoglein antibodies in blood. An elevated level of this antibody can help confirm the presence of pemphigus
• Blood tests, analysis of pus, and culture of the fluid being drained may be undertaken, as needed, to check for any infections
• Dermoscopy: Dermoscopy is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
• Immunofluorescence studies on the skin biopsy specimen

A differential diagnosis may be necessary to eliminate other conditions that present similar signs and symptoms such as:

• Atopic dermatitis
• Cutaneous lupus erythematosus
• Erythrodermic psoriasis
• Pemphigus vulgaris
• Seborrheic dermatitis
• Staphylococcal scalded skin syndrome

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Fogo Selvagem?

The possible complications due to Fogo Selvagem include:

• Severe emotional stress
• Cosmetic issues
• Superimposed bacterial and fungal skin infections
• Complications from a lack of prompt treatment may include:
  • Growth delays in children
  • Dwarfism
  • Male infertility
  • Increased susceptibility to viral infections
• Treatment complications and medication side effects

How is Fogo Selvagem Treated?

The treatment measures available for Fogo Selvagem include:

• Topical and systemic corticosteroids are the mainstay of therapy
• If necessary, systemic immunosuppressant medications may be prescribed
• Undertaking treatment of any secondary infections through administration of antibiotic or antifungal agents
• Good skin care and general body hygiene

Regular follow up visits with the healthcare provider is important and recommended.

How can Fogo Selvagem be Prevented?

It may not be possible to currently prevent Fogo Selvagem; however, the risk for the same may be decreased by considering the following steps:

• Creating an awareness of the skin condition in the endemic regions
• Maintaining basic personal/community hygiene and proper sanitation is highly important, particularly in the endemic zones
• Using insect repellents to prevent insects (such as black flies) from entering the residence areas

The growth of flies must be effectively controlled and methods for eradication followed on a regular basis.

What is the Prognosis of Fogo Selvagem? (Outcomes/Resolutions)

The prognosis of Fogo Selvagem is generally good with prompt and adequate treatment.

• An early appropriate treatment can help avoid long-term complications and ensure faster healing
• The prognosis is reportedly better than that of pemphigus vulgaris, which is the most common form of pemphigus

Additional and Relevant Useful Information for Fogo Selvagem:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/