Fogo Selvagem Treatment & Management

Updated: Jun 13, 2016
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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Treatment

Medical Care

Mario Fonzari pioneered the use of cortisone and thereby revolutionized treatment of this disorder, substantially reducing mortality. [29, 30] For patients with severe fogo selvagem, systemic corticosteroid therapy is the treatment of choice. Prednisone (1 mg/kg body weight) is administered as a single daily morning dose until blister formation ceases or the Nikolsky sign disappears. After initial control is achieved, the prednisone dose is reduced to about half the initial dose. This reduction is followed by slow tapering to the minimal effective maintenance dose over weeks to months. Adjuvant therapy includes the use of immunosuppressants such as azathioprine (1-2 mg/kg body weight until lesions clear; with slow tapering of dose), cyclophosphamide (100-200 mg qd, with a reduction to a maintenance dose of 50-100 mg qd), and mycophenolate mofetil (1.5-3 g/d).

Therapy for fogo selvagem is usually less aggressive than that of pemphigus vulgaris because of the lower morbidity and mortality rates. In patients with limited involvement, topical glucocorticosteroids may be sufficient. In more extensive cases, an aggressive treatment (similar to that of pemphigus vulgaris) is necessary; this treatment includes systemic corticosteroids and immunosuppressants. In some cases, coadministration of anti-inflammatory agents such as gold, antimalarials, sulfones, or antibiotics may be useful. In some patients with pemphigus foliaceus, nicotinamide 1.5 g/d and tetracycline 2 g/d is reported to be useful.

Topical treatment with antibiotics and corticosteroids is beneficial. Potent corticosteroid ointments may be effective with long-lasting lesions.

In some patients, photoprotection may be helpful because UV-B light may trigger acantholysis and cause the disease to flare.

Antiacantholytic therapies in patients with pemphigus, using cholinomimetics, indicates that cholinergic drugs may be a promising approach for fogo selvagem. [31]

Plasmapheresis is another therapeutic option. Plasmapheresis is indicated for patients with poorly controlled disease or those with high circulating autoantibody titers. In some patients, this therapy may decrease the autoantibody titers and favorably influence the clinical outcome, especially in otherwise therapy-resistant pemphigus foliaceus. Plasmapheresis should be used in conjunction with daily cyclophosphamide treatment to reduce a predictable rebound increase in autoantibody synthesis. Potential complications, including the need for maintaining venous access, a bleeding tendency, electrolyte shifts, pulmonary edema, fever, chills, hypotension, and septicemia, should be considered.

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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. [21] Residing in a rural area may also be a risk factor for complications.

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Prevention

In endemic areas, measures to minimize exposure to biting flies and other insect vectors are appropriate. These measures include the use of insecticides, protective clothing, and insect screens. In some patients, photoprotection may be helpful because UV-B light may trigger acantholysis and cause the disease to flare.

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Long-Term Monitoring

Frequent clinical follow-up is required in patients with active disease. Patients should be monitored for disease progression, development of complications such as infections, and compliance with and complications of their medications. Some clinicians monitor indirect IF titers to gauge the response to therapy; however, this practice is not universal. Patients taking long-term steroids and/or immunosuppressants should be appropriately followed up and monitored. The lowest possible dose of steroids and immunosuppressants should be used to minimize the potential for systemic toxicities.

Be aware of bacterial, viral, or fungal secondary infections and infestations. Disseminated strongyloidiasis is reported.

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