Fogo Selvagem Clinical Presentation

Updated: Apr 16, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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Presentation

History

Patients may describe the burning sensation for which the disorder is named. Bullae usually begin on the head, neck, and trunk in a seborrheic distribution. Patients may experience an intense burning or itching sensation.

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Physical Examination

The clinical features of fogo selvagem are identical to those in the classic form of pemphigus foliaceus (Cazenave). The primary cutaneous lesions are superficial vesicles or blisters that rupture easily and leave superficial erosions.

The Nikolsky sign (ie, epidermal detachment after pressure on perilesional and nonlesional skin) usually is spread acrally. The Nikolsky sign emphasizes that physical trauma can shear the pathologic epidermis in pemphigus foliaceus to produce clinical lesions. The erosions can become numerous and have a tendency to generalize. Note the image below.

Generalized form with blisters and crusts on the t Generalized form with blisters and crusts on the trunk.

In contrast to pemphigus vulgaris, pemphigus foliaceus involves little or no involvement of the mucous membranes. UV exposure may enhance lesions or trigger their development.

Localized or generalized forms exist. Generalized forms may evolve into exfoliative erythroderma and lead to death (rare). Occasionally, extensive exfoliative erythroderma develops. Hyperpigmented or verrucous lesions may be observed in the course of the disease.

The primary lesions are small superficial blisters. These flaccid bullae are difficult to find because they are transient and transform into erosions.

The lesions tend to appear on the central trunk and face (ie, face, scalp, upper trunk) in a distribution similar to that of seborrheic dermatitis. Sometimes, atrophic changes of the nails and hair are evident. Burning and local pain may occur with the erosions. In some patients, a herpetiform variant is also observed.

Some patients with pemphigus foliaceus have the initial features of pemphigus herpetiformis. In Tunisia, this feature occurs in 35% of patients with endemic pemphigus foliaceus.

A variant of endemic pemphigus foliaceus in Colombia, South America is characterized by palms and soles that have an edematous texture and mild hyperkeratosis in addition to the blisters on nonglabrous skin. [31]

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Complications

Fogo selvagem itself and its therapy may both cause difficulties for the patient. The major complication of fogo selvagem is impetigo. Secondary fungal infections, Kaposi varicelliform eruption, scabies, and disseminated strongyloidiasis may occur.

Other complications reported in patients with fogo selvagem include dwarfism, azoospermia, dermatophytosis, warts, and Norwegian scabies. These complications may be related to long-term corticosteroid treatment.

In Peru, complications such as pyodermitis and pyelonephritis were found to occur during treatment, with increased risk in those noncompliant with treatment and in those having the generalized clinical form. [30] Residing in a rural area may also be a risk factor for complications.

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