Medscape is available in 5 Language Editions – Choose your Edition here.


Fogo Selvagem Treatment & Management

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

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.



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.



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.


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.

Contributor Information and Disclosures

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Mark G Lebwohl, MD Chairman, Department of Dermatology, Mount Sinai School of Medicine

Mark G Lebwohl, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Received none from Amgen for consultant & investigator; Received none from Novartis for consultant & investigator; Received none from Pfizer for consultant & investigator; Received none from Celgene Corporation for consultant & investigator; Received none from Clinuvel for consultant & investigator; Received none from Eli Lilly & Co. for consultant & investigator; Received none from Janssen Ortho Biotech for consultant & investigator; Received none from LEO Pharmaceuticals for consultant & inves.

  1. Nikolsky PV. Materiali K. uchenigu o pemphigus foliaceus [doctoral thesis]. Kiev. 1896.

  2. Bastuji-Garin S, Souissi R, Blum L, et al. Comparative epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol. 1995 Feb. 104(2):302-5. [Medline].

  3. Ribeiro JM, Valenzuela JG, Pham VM, Kleeman L, Barbian KD, Favreau AJ, et al. An insight into the sialotranscriptome of Simulium nigrimanum, a black fly associated with fogo selvagem in South America. Am J Trop Med Hyg. 2010 Jun. 82(6):1060-75. [Medline]. [Full Text].

  4. Culton DA, Qian Y, Li N, et al. Advances in pemphigus and its endemic pemphigus foliaceus (Fogo Selvagem) phenotype: a paradigm of human autoimmunity. J Autoimmun. 2008 Dec. 31(4):311-24. [Medline].

  5. Flores G, Qian Y, Díaz LA. The enigmatic autoimmune response in endemic pemphigus foliaceus. Actas Dermosifiliogr. 2009 Dec. 100 Suppl 2:40-8. [Medline].

  6. Aoki V, Rivitti EA, Diaz LA, Cooperative Group on Fogo Selvagem Research. Update on fogo selvagem, an endemic form of pemphigus foliaceus. J Dermatol. 2015 Jan. 42 (1):18-26. [Medline].

  7. Gonzalez F, Saenz AM, Cirocco A, Tacaronte IM, Fajardo JE, Calebotta A. Endemic pemphigus foliaceus in Venezuela: report of two children. Pediatr Dermatol. 2006 Mar-Apr. 23(2):132-5. [Medline].

  8. Aoki V, Sousa JX Jr, Diaz LA. Pathogenesis of endemic pemphigus foliaceus. Dermatol Clin. 2011 Jul. 29(3):413-8, viii. [Medline].

  9. Qian Y, Jeong JS, Abdeladhim M, Valenzuela JG, Aoki V, Hans-Filhio G, et al. IgE anti-LJM11 sand fly salivary antigen may herald the onset of fogo selvagem in endemic Brazilian regions. J Invest Dermatol. 2015 Mar. 135 (3):913-5. [Medline].

  10. Aoki V, Millikan RC, Rivitti EA, et al. Environmental risk factors in endemic pemphigus foliaceus (fogo selvagem). J Investig Dermatol Symp Proc. 2004 Jan. 9(1):34-40. [Medline].

  11. Robledo MA. Chronic methyl mercury poisoning may trigger endemic pemphigus foliaceus "fogo selvagem". Med Hypotheses. 2012 Jan. 78(1):60-6. [Medline].

  12. Diaz LA, Arteaga LA, Hilario-Vargas J, et al. Anti-desmoglein-1 antibodies in onchocerciasis, leishmaniasis and Chagas disease suggest a possible etiological link to Fogo selvagem. J Invest Dermatol. 2004 Dec. 123(6):1045-51. [Medline].

  13. Rodrigues DB, Pereira SA, dos Reis MA, et al. In situ detection of inflammatory cytokines and apoptosis in pemphigus foliaceus patients. Arch Pathol Lab Med. 2009 Jan. 133(1):97-100. [Medline].

  14. Qian Y, Prisayanh P, Andraca E, et al. IgE, IgM, and IgG4 Anti-Desmoglein 1 Autoantibody Profile in Endemic Pemphigus Foliaceus (Fogo Selvagem). J Invest Dermatol. 2010 Dec 30. [Medline].

  15. Qian Y, Jeong JS, Maldonado M, Valenzuela JG, Gomes R, Teixeira C, et al. Cutting Edge: Brazilian pemphigus foliaceus anti-desmoglein 1 autoantibodies cross-react with sand fly salivary LJM11 antigen. J Immunol. 2012 Aug 15. 189(4):1535-9. [Medline]. [Full Text].

  16. Qian Y, Jeong JS, Ye J, Dang B, Abdeladhim M, Aoki V, et al. Overlapping IgG4 Responses to Self- and Environmental Antigens in Endemic Pemphigus Foliaceus. J Immunol. 2016 Mar 1. 196 (5):2041-50. [Medline].

  17. Abreu-Velez AM, Howard MS, Yi H, Gao W, Hashimoto T, Grossniklaus HE. Neural System Antigens Are Recognized by Autoantibodies from Patients Affected by a New Variant of Endemic Pemphigus Foliaceus in Colombia. J Clin Immunol. 2011 Jan 6. [Medline].

  18. Hernandez-Perez E. Pemphigus in El Salvador. An eight-year study (1970-1977). Int J Dermatol. 1979 Oct. 18(8):645-8. [Medline].

  19. Ortega-Loayza AG, Ramos W, Gutierrez EL, Jimenez G, Rojas I, Galarza C. Endemic pemphigus foliaceus in the Peruvian Amazon. Clin Exp Dermatol. 2013 Aug. 38(6):594-600. [Medline].

  20. Morini JP, Jomaa B, Gorgi Y, et al. Pemphigus foliaceus in young women. An endemic focus in the Sousse area of Tunisia. Arch Dermatol. 1993 Jan. 129(1):69-73. [Medline].

  21. Ramos W, Chacon GR, Galarza C, Gutierrez EL, Smith ME, Ortega-Loayza AG. Endemic pemphigus in the Peruvian Amazon: epidemiology and risk factors for the development of complications during treatment. An Bras Dermatol. 2012 Nov-Dec. 87(6):838-45. [Medline]. [Full Text].

  22. Abreu Velez AM, Howard MS, Hashimoto T. Palm tissue displaying a polyclonal autoimmune response in patients affected by a new variant of endemic pemphigus foliaceus in Colombia, South America. Eur J Dermatol. 2010 Jan-Feb. 20(1):74-81. [Medline].

  23. Beutner EH, Prigenzi LS, Hale W, Leme Cde A, Bier OG. Immunofluorescent studies of autoantibodies to intercellular areas of epithelia in Brazilian pemphigus foliaceus. Proc Soc Exp Biol Med. 1968 Jan. 127(1):81-6. [Medline].

  24. Ambiel MV, Roselino AM. Prevalence of Metabolic Syndrome and its components in a Brazilian sample of pemphigus patients. An Bras Dermatol. 2014 Sep. 89(5):752-6. [Medline]. [Full Text].

  25. Friedman H, Campbell IT, Alvarez RR, et al. [Indirect immunofluorescence in endemic pemphigus foliaceus. A contribution to its standardization]. Rev Inst Med Trop Sao Paulo. 1989 May-Jun. 31(3):158-68. [Medline].

  26. Diaz LA, Prisayanh PS, Dasher DA, et al. The IgM anti-desmoglein 1 response distinguishes Brazilian pemphigus foliaceus (fogo selvagem) from other forms of pemphigus. J Invest Dermatol. 2008 Mar. 128(3):667-75. [Medline].

  27. Cunha PR, Bystryn JC, Medeiros EP, de Oliveira JR. Sensitivity of indirect immunofluorescence and ELISA in detecting intercellular antibodies in endemic pemphigus foliaceus (Fogo Selvagem). Int J Dermatol. 2006 Aug. 45(8):914-8. [Medline].

  28. Qaqish BF, Prisayanh P, Qian Y, et al. Development of an IgG4-based predictor of endemic pemphigus foliaceus (fogo selvagem). J Invest Dermatol. 2009 Jan. 129(1):110-8. [Medline].

  29. Chacón GR, Ortega-Loayza AG, Cyr RM. Historical notes on endemic pemphigus in South America. Int J Dermatol. 2012 Apr. 51(4):477-81. [Medline].

  30. Fonzari M. [Combination of cortisone derivatives and antibiotics in pemphigus]. Rev Paul Med. 1962 Jul. 61:53-6. [Medline].

  31. Grando SA. Cholinergic control of epidermal cohesion. Exp Dermatol. 2006 Apr. 15(4):265-82. [Medline].

  32. Fonzari M. Ensaios terapeuticos no penfigo foliaceo. Arquivos Dermatologia Sifiligrafia Sao Paulo. 1952. 14:10-34.

  33. Vieira JP. Novas contribuicoes ao estudo do penfigo foliaceo (fogo-selvagem) no estado de Sao Paulo. Empresa Grafica da Revista dos Tribunais Sao Paulo, Brazil. 1940. 1-242.

  34. Vieira JP. Penfigo foliaceo e syndromo de Senear-Uscher. Empresa Grafica da Revista dos Tribunais Sao Paulo, Brazil. 1942. 1-171.

  35. Zilberberg B. Penfigo e dermatite de Duhring-Brocq. Contributicao papa o seu estudo cito-histologico. Arquivos Dermatologia Sifiligrafia Sao Paulo. 1954. 16:43-89.

Generalized form with blisters and crusts on the trunk.
Immunoprecipitation with the recombinant desmoglein 1. Lane 1 is the result in normal human serum. Lanes 2-7 are findings in sera from patients with fogo selvagem. Lanes 2-7 show the reaction of the sera against the extracellular domain of desmoglein 1.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.