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Fogo selvagem

Author: James Cameron, Medical Student, University of Auckland. DermNet New Zealand Editor in Chief: Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. 

What is fogo selvagem?

Fogo selvagem, otherwise known as endemic pemphigus foliaceus, is an autoimmune blistering disease. It is a variant of pemphigus foliaceus with a unique epidemiological profile.

Who gets fogo selvagem?

Fogo selvagem is most prevalent in rural areas of the southern and midwestern states of Brazil, such as Goiás, Mato Grosso do Sul, Paraná, Sao Paulo, and Minas Gerais. Cases have also been reported in Tunisia, Peru, Paraguay, El Salvador and Colombia. It most commonly affects children and young adults.

Fogo selvagem is closely associated with poverty and malnutrition, and has a higher incidence in areas near rivers and streams. Fogo selvagem has associations with the human leukocyte antigen complex (HLA), indicating a genetic predisposition to the disease.

What causes fogo selvagem?

Fogo selvagem is an autoimmune disease in which an individual's immune system reacts against the keratinocytes and desmosomes that make up the skin.

In reaction to fogo selvagem, the body produces IgG4 antibodies which bind to the desmosomal protein desmoglein-1 (Dsg1). This causes the surface keratinocytes of the epidermis to detach from each other and fill with fluid, causing the characteristic blisters.

The exact pathogenesis of fogo selvagem is not known, but a combination of genetic and environmental factors are thought to be involved. Expression of human leukocyte antigen (HLA)-DRB1- 0404, 1402 or 1406 alleles is found in patients with fogo selvagem, suggesting genetic susceptibility. It is hypothesised that a salivary protein from a blood-sucking insect like the black fly (Simulium nigrimanum) triggers the autoimmune response, as black fly bites were found to be more common in patients with fogo selvagem.   

What are the clinical features of fogo selvagem? 

The classic lesions of fogo selvagem are superficial blisters or vesicles that progress over weeks to months.

What are the complications of fogo selvagem?

If untreated, complications may include:

These complications are less likely to occur if the disease is treated immediately.

How is fogo selvagem diagnosed?

Diagnosis of fogo selvagem is based on local prevalence of the disease and characteristic blisters with a positive Nikolsky sign in which gently rubbing affected skin causes a blister to form.

Skin biopsy shows acantholysis of the upper epidermis, often resulting in a subcorneal cleft. The subcorneal cleft increases in size and can detach, with or without bulla formation. Other histological findings can include papillomatosis, hyperkeratosis and parakeratosis. Mild lymphocytic infiltration may be present.

Direct immunofluorescence demonstrates epidermal intercellular fluorescence, mainly anti-IgG or anti-C3 autoantibodies.

ELISA (rDsg1-His) has high sensitivity in detecting intercellular fogo selvagem autoantibodies.

What is the differential diagnosis for fogo selvagem?

Other skin conditions may be under consideration in a patient with superficial blistering of the scalp, face, neck and upper trunk such as:

For generalised blistering, consider:

What is the treatment for fogo selvagem?

Fogo selvagem is treated with systemic corticosteroids. such as prednisone or prednisolone, to which most patients respond well. Immune suppressive agents may be added in, such as:

What is the outcome for fogo selvagem?

With adequate treatment, the prognosis of fogo selvagem is good with no long-term sequelae.

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