eMedicine Specialties > Dermatology > Bullous Diseases

Pemphigus Foliaceus: Treatment & Medication

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Slawomir Majewski, MD, Professor and Director, Department of Dermatology and Venereology, Warsaw School of Medicine, Poland; Sebastian S Majewski, MD, Consulting Staff, Department of Dermatology, Military Institute of Health Services, Warsaw, Poland
Contributor Information and Disclosures

Updated: May 5, 2009

Treatment

Medical Care

Present information is probably inadequate to ascertain the optimal therapy for pemphigus foliaceus (PF), including the optimal glucocorticoid dose, the role of adjuvant immunosuppressive medications, and long-term adverse events to improve the risk-to-benefit ratio.30 Therapy for pemphigus foliaceus is usually less aggressive than that of PV because of lower morbidity and mortality rates.31
 
First results indicate that nonsteroidal treatment of pemphigus is possible. Mestinon may be used to slow down progression of the disease and to treat mild cases with chronic lesions on limited areas. Antimalarial therapy may be effective monotherapy in some patients. However, a major obstacle in comparing therapeutic outcomes is the lack of generally accepted definitions and measurements for the clinical evaluation of patients with pemphigus.32 Common terms and endpoints of pemphigus are needed to accurately measure and assess disease extent, activity, severity, and therapeutic response.

  • Topical glucocorticosteroids may be sufficient in cases of limited involvement.33
  • In more extensive cases (similar to PV), adjuvant immunosuppressants, including systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclophosphamide, and cyclosporin A, may be necessary.34,35
  • In some cases, such as PE, combined therapy is beneficial with the use of corticosteroids and sulfones or antimalarial agents.
  • Topical treatment with antibiotics and corticosteroids, such as topical clobetasol cream or ointment 0.05% twice a day, is helpful. Other vehicles that may be useful are creams, foams, liquids (for scalp lesions), and aerosols. Antibiotics, such as minocycline 50 mg daily, may be effective. Nicotinamide 1.5 g/d and tetracycline 2 g/d have also been reported to be beneficial in a small number of patients. Antibiotics and nicotinamide are purported to have anti-inflammatory effects.36
  • Photoprotection is appropriate for some patients because UV-B may trigger acantholysis and cause a flare-up of the disease.
  • Successful anti-CD20 antibody treatment has also been described.37
  • Plasmapheresis is another therapeutic option in patients with recalcitrant disease. It may decrease autoantibody titers in some patients and favorably influence the clinical outcome, especially in patients with otherwise therapy-resistant pemphigus foliaceus. It is often used in conjunction with cytostatic agents, such as cyclophosphamide or azathioprine, 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.
  • Amagai et al reported that a single cycle of intravenous immunoglobulin at 400 mg/kg/d for 5 days is effective and safe for patients with pemphigus that is relatively resistant to systemic steroid therapy.38 Toth and Jonkman also reported on successful therapy with intravenous immunoglobulin (low dose).39

Medication

A number of medications are used to treat patients with pemphigus foliaceus. They are often used in combination. Refractory pemphigus foliaceus as been treated with the anti-CD20 monoclonal antibody rituximab.40,41

Corticosteroid agents

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.


Prednisone (Deltasone, Orasone)

Synthetic adrenocortical steroid with predominantly glucocorticoid properties. Immunosuppressant for the treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.

Adult

60-100 mg PO every morning or more often as required to abort acantholysis; alternatively, 0.5-2 mg/kg/d PO; taper as condition improves; single morning dose is safer for long-term use, but divided doses have more anti-inflammatory effect

Pediatric

0.14-2 mg/kg/d PO divided tid/qid (4-60 mg/m2/d)

Coadministration with estrogens may decrease clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase the metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics; coadministration with ritonavir may significantly increase serum concentrations of prednisone; concomitant therapy with montelukast may result in severe peripheral edema; clarithromycin may increase risk of psychotic symptoms

Documented hypersensitivity; viral, fungal, tubercular skin, or connective tissue infections; peptic ulcer disease; hepatic dysfunction; GI disease

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

May unmask hypertension or diabetes or exacerbate peptic ulcer disease and tuberculosis; long-term sequelae associated with long-term steroid use include osteoporosis, cataracts, and pituitary-hypothalamic axis suppression; with high doses, patients may develop a steroid psychosis and are at increased risk of infections, particularly when oral steroids are used in conjunction with other immunosuppressants; frequently monitor patient's blood glucose level, blood pressure, and weight; monitor for Cushing syndrome

Antibiotic agents

Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.


Minocycline (Dynacin, Minocin)

Semisynthetic derivative of tetracycline. Treats infections caused by susceptible gram-negative and gram-positive organisms, in addition to infections caused by susceptible Chlamydia, Rickettsia, and Mycoplasma species. Was found to be effective in some nontuberculotic mycobacterial infections.

Adult

50-100 mg PO bid

Pediatric

<8 years: Not recommended
>8 years: 4 mg/kg PO initially, followed by 2 mg/kg q12h

Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; can decrease effects of PO contraceptives, causing breakthrough bleeding and increased risk of pregnancy; tetracyclines can increase hypoprothrombinemic effects of anticoagulants; concomitant use of vitamin A supplementation or oral retinoids (eg, isotretinoin) not recommended; additive risk of pseudotumor cerebri; simultaneous administration of cinnamon and tetracycline may slow tetracycline absorption; bacteriostatic drugs (eg, tetracyclines) may interfere with bactericidal effect of penicillin

Documented hypersensitivity; severe hepatic dysfunction

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Photosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; tetracycline use during tooth development (last half of pregnancy through age 8 y) can cause permanent discoloration of teeth; Fanconilike syndrome may occur with outdated tetracyclines; hepatitis or lupuslike syndromes may occur; autoimmune syndromes; drug-induced lupus–like syndrome, hepatitis, and vasculitis reported with long-term use; caution in preexisting renal impairment; risk of azotemia, hyperphosphatemia, and acidosis due to drug accumulation; minocycline use may result in false elevations of urinary catecholamine levels due to interference with the fluorescence test


Dapsone (Avlosulfon)

Bactericidal and bacteriostatic against mycobacteria; mechanism of action is similar to that of sulfonamides where competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. Used to control the dermatologic symptoms of dermatitis herpetiformis. Can be used for patients with pemphigus and may be DOC for PH and IgA PF. May be provided as monotherapy or in combination with systemic steroids and immunosuppressants.

Adult

50-200 mg PO qd

Pediatric

Not established

May inhibit anti-inflammatory effects of clofazimine; hematologic reactions may increase with folic acid antagonists, eg, pyrimethamine (monitor for agranulocytosis during second and third months of therapy); probenecid increases toxicity; trimethoprim with dapsone may increase toxicity of both drugs; because of increased renal clearance, levels may significantly decrease when administered concurrently with rifampin

Documented hypersensitivity; known G-6-PD deficiency (assay for G-6-PD activity prior to initiation of therapy)

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Associated with a variety of systemic toxicities, including agranulocytosis, anemia, methemoglobinemia, hepatitis, and neuropathy; patients may experience headache and/or GI distress on initiation of therapy; perform weekly blood counts (first mo), then monthly WBC counts (6 mo), then semiannual WBC counts; discontinue if a significant reduction in platelets, leukocytes, or hematopoiesis occurs; caution in methemoglobin reductase deficiency, G-6-PD deficiency, or hemoglobin M because of high risk for hemolysis and Heinz body formation; caution in patients exposed to other agents or conditions (eg, infection, diabetic ketosis) capable of producing hemolysis; peripheral neuropathy can occur (rare); phototoxicity may occur when exposed to UV light; pancreatitis may occur; various forms of renal complications including acute renal failure, acute tubular necrosis, and oliguria have occurred with dapsone use

Antimalarial agents

Hydroxychloroquine has immunosuppressive effects.


Hydroxychloroquine (Plaquenil)

4-Aminoquinoline derivative active against a variety of autoimmune disorders. Inhibits chemotaxis of eosinophils, locomotion of neutrophils, and impairs complement-dependent antigen-antibody reactions. Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate.

Adult

400 mg PO qd or divided bid (mg/kg same as pediatric dosing)

Pediatric

Not to exceed 6.5 mg/kg/d PO

May increase penicillamine levels; serum levels of hydroxychloroquine may increase with cimetidine; magnesium trisilicate may decrease absorption; concurrent use of aurothioglucose and antimalarial agents may induce blood dyscrasias and may also result in additive risk of this effect; concurrent digoxin may result in increased serum digoxin concentrations

Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Crosses placenta and may cause ocular, CNS, or ototoxicity in fetus; do not use in breastfeeding; limit pediatric use to established safe doses to avoid potential fatality; perform regular ophthalmologic examinations (including visual acuity, slit lamp, funduscopic, and visual-field tests); caution in patients with G-6-PD deficiency; check blood cell counts periodically (perhaps biannually)
Hemolysis, aplastic anemia, agranulocytosis, and leukopenia can occur; not recommended for long-term use in children; perform periodic (6 mo) ophthalmologic examinations; test periodically for muscle weakness

Immunomodulatory agents

These agents have antiproliferative and immunosuppressive effects.


Azathioprine (Imuran)

May be used alone or as steroid-sparing agent. Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.

Adult

100-200 mg PO qd in combination with prednisone; alternatively, 1 mg/kg/d PO for 6-8 wk; increase by 0.5 mg/kg q4wk until response or dose reaches 2.5 mg/kg/d

Pediatric

Not established

Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine; coadministration with mycophenolate may increase toxicity; alfalfa, black Cohosh, and echinacea may reduce immunosuppressive drug effectiveness

Documented hypersensitivity; deficiency of thiopurine methyltransferase (can result in severe myelosuppression and leukopenia); history of treatment with alkylating agents

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Adverse effects include teratogenicity, hepatitis, bone marrow suppression, and increased risk of cancer; before initiating therapy and regularly thereafter, perform urine analysis, complete blood cell count, renal and liver function tests, and serum electrolyte levels; increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur


Cyclophosphamide (Cytoxan, Neosar)

May be used as monotherapy or as a steroid-sparing agent. Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

Adult

50-100 mg IV qd in combination with prednisone; 2.5-3 mg/kg/d PO divided qid

Pediatric

Not established

Allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones; chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting cholinesterase activity; concurrent use of NSAIDs has resulted in increases in cyclosporine levels, nephrotoxicity, and increased plasma creatinine concentrations; concomitant use of ACE inhibitors may decrease renal function; coadministration with nevirapine and St. John's wort may reduce immunosuppressive drug effectiveness
Increased risk of infection by live vaccine; coadministration with trastuzumab may increase cardiac toxicity; coadministration with tamoxifen may increase risk of thromboembolism

Documented hypersensitivity; severely depressed bone marrow function

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis; adverse effects include oligospermia or azoospermia, cardiomyopathy, infectious disease, interstitial pneumonia, increase risk of malignancy, possibility of increased toxicity in adrenalectomized patients

More on Pemphigus Foliaceus

Overview: Pemphigus Foliaceus
Differential Diagnoses & Workup: Pemphigus Foliaceus
Treatment & Medication: Pemphigus Foliaceus
Follow-up: Pemphigus Foliaceus
Multimedia: Pemphigus Foliaceus
References
[ CLOSE WINDOW ]

References

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

  2. Chorzelski T, Jablonska S, Blaszczyk M. Immunopathological investigations in the Senear-Usher syndrome (coexistence of pemphigus and lupus erythematosus). Br J Dermatol. Apr 1968;80(4):211-7. [Medline].

  3. Jablonska S, Chorzelski TP, Beutner EH, Chorzelska J. Herpetiform pemphigus, a variable pattern of pemphigus. Int J Dermatol. Jun 1975;14(5):353-9. [Medline].

  4. Abreu-Velez AM, Hashimoto T, Bollag WB, et al. A unique form of endemic pemphigus in northern Colombia. J Am Acad Dermatol. Oct 2003;49(4):599-608. [Medline].

  5. Abreu-Velez AM, Beutner EH, Montoya F, Bollag WB, Hashimoto T. Analyses of autoantigens in a new form of endemic pemphigus foliaceus in Colombia. J Am Acad Dermatol. Oct 2003;49(4):609-14. [Medline].

  6. Chorzelski TP, Hashimoto T, Amagai M, et al. Paraneoplastic pemphigus with cutaneous and serological features of pemphigus foliaceus. Br J Dermatol. Aug 1999;141(2):357-9. [Medline].

  7. Ishii K, Amagai M, Komai A, et al. Desmoglein 1 and desmoglein 3 are the target autoantigens in herpetiform pemphigus. Arch Dermatol. Aug 1999;135(8):943-7. [Medline].

  8. Kubo A, Amagai M, Hashimoto T, et al. Herpetiform pemphigus showing reactivity with pemphigus vulgaris antigen (desmoglein 3). Br J Dermatol. Jul 1997;137(1):109-13. [Medline].

  9. Masmoudi A, Baricault S, Chikrouhou H, et al. [Tunisian pemphigus foliaceus with antidesmoglein 3 antibody]. Ann Dermatol Venereol. Jan 2008;135(1):69-70. [Medline].

  10. Wu H, Wang ZH, Yan A, et al. Protection against pemphigus foliaceus by desmoglein 3 in neonates. N Engl J Med. Jul 6 2000;343(1):31-5. [Medline].

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

  12. Ruocco V, Ruocco E. Pemphigus and environmental factors. Giornale Dermatol Venereol. 2003;138:299-309.

  13. Ruocco V. Attualita' sul pemfigo indotto. Capri, Italy: 2003. 41st Italian National Dermatology Congress.

  14. Pigozzi B, Peserico A, Schiesari L, Alaibac M. Pemphigus foliaceus evolving into pemphigus vulgaris: a probable example of 'intermolecular epitope spreading' confirmed by enzyme-linked immunosorbent assay study. J Eur Acad Dermatol Venereol. Feb 2008;22(2):242-4. [Medline].

  15. 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. Feb 1995;104(2):302-5. [Medline].

  16. Warren SJ, Lin MS, Giudice GJ, et al. The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. Cooperative Group on Fogo Selvagem Research. N Engl J Med. Jul 6 2000;343(1):23-30. [Medline].

  17. Metry DW, Hebert AA, Jordon RE. Nonendemic pemphigus foliaceus in children. J Am Acad Dermatol. Mar 2002;46(3):419-22. [Medline].

  18. Daoud YJ, Foster CS, Ahmed R. Eyelid skin involvement in pemphigus foliaceus. Ocul Immunol Inflamm. Sep-Oct 2005;13(5):389-94. [Medline].

  19. Uzun S, Durdu M. The specificity and sensitivity of Nikolskiy sign in the diagnosis of pemphigus. J Am Acad Dermatol. Mar 2006;54(3):411-5. [Medline].

  20. Brenner S, Ruocco V. D-penicillamine-induced pemphigus foliaceus with autoantibodies to desmoglein-1. J Am Acad Dermatol. Jul 1998;39(1):137-8. [Medline].

  21. Olszewska M, Misiewiz J, Kolakowska-Starzyk H. Penicillamine-induced pemphigus herpetiformis (foliaceus). Dermatol Klin (Wroclaw). 2001;3 (Suppl 1):101.

  22. Lin R, Ladd DJ Jr, Powell DJ, Way BV. Localized pemphigus foliaceus induced by topical imiquimod treatment. Arch Dermatol. Jul 2004;140(7):889-90. [Medline].

  23. Fujita H, Iguchi M, Watanabe R, Asahina A. Pemphigus foliaceus induced by bucillamine. Eur J Dermatol. Jan-Feb 2007;17(1):98-9. [Medline].

  24. Kraigher O, Wohl Y, Gat A, Brenner S. A mixed immunoblistering disorder exhibiting features of bullous pemphigoid and pemphigus foliaceus associated with Spirulina algae intake. Int J Dermatol. Jan 2008;47(1):61-3. [Medline].

  25. Jarzabek-Chorzelska M, Jablonska S, Kolacinska-Strasz Z, Sulej I. Immunopathological diagnosis of pemphigus foliaceus. Dermatology. 2002;205(4):413-5; author reply 415-6; discussion 416. [Medline].

  26. Harman KE, Gratian MJ, Seed PT, Bhogal BS, Challacombe SJ, Black MM. Diagnosis of pemphigus by ELISA: a critical evaluation of two ELISAs for the detection of antibodies to the major pemphigus antigens, desmoglein 1 and 3. Clin Exp Dermatol. May 2000;25(3):236-40. [Medline].

  27. Martel P, Gilbert D, Labeille B, Kanitakis J, Joly P. A case of paraneoplastic pemphigus with antidesmoglein 1 antibodies as determined by immunoblotting. Br J Dermatol. Apr 2000;142(4):812-3. [Medline].

  28. Narbutt J, Sysa-Jedrzejowska A, Torzecka JD. The usefulness of enzyme-linked immunosorbent assay for serodiagnosis of pemphigus vulgaris and pemphigus foliaceus at various stages of diseaseactivity. Przegl Dermatol. 2002;89:447-452.

  29. Nagao K, Tanikawa A, Yamamoto N, Amagai M. Decline of anti-desmoglein 1 IgG ELISA scores by withdrawal of D-penicillamine in drug-induced pemphigus foliaceus. Clin Exp Dermatol. Jan 2005;30(1):43-5. [Medline].

  30. Martin LK, Werth V, Villanueva E, Segall J, Murrell DF. Interventions for pemphigus vulgaris and pemphigus foliaceus. Cochrane Database Syst Rev. Jan 21 2009;CD006263. [Medline].

  31. Grando SA. New approaches to the treatment of pemphigus. J Investig Dermatol Symp Proc. Jan 2004;9(1):84-91. [Medline].

  32. Murrell DF, Dick S, Ahmed AR, et al. Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus. J Am Acad Dermatol. Jun 2008;58(6):1043-6. [Medline].

  33. Dumas V, Roujeau JC, Wolkenstein P, Revuz J, Cosnes A. The treatment of mild pemphigus vulgaris and pemphigus foliaceus with a topical corticosteroid. Br J Dermatol. Jun 1999;140(6):1127-9. [Medline].

  34. Gupta R. Prolonged remission of pemphigus induced by dexamethasone-cyclophosphamide pulse therapy. Indian J Dermatol Venereol Leprol. Mar-Apr 2007;73(2):121-2. [Medline].

  35. Katz KH, Marks JG Jr, Helm KF. Pemphigus foliaceus successfully treated with mycophenolate mofetil as a steroid-sparing agent. J Am Acad Dermatol. Mar 2000;42(3):514-5. [Medline].

  36. Chaffins ML, Collison D, Fivenson DP. Treatment of pemphigus and linear IgA dermatosis with nicotinamide and tetracycline: a review of 13 cases. J Am Acad Dermatol. Jun 1993;28(6):998-1000. [Medline].

  37. Herr AL, Hatami A, Kokta V, Dalle JH, Champagne MA, Duval M. Successful anti-CD20 antibody treatment of pemphigus foliaceus after unrelated cord blood transplantation. Bone Marrow Transplant. Feb 2005;35(4):427-8. [Medline].

  38. [Best Evidence] Amagai M, Ikeda S, Shimizu H, et al. A randomized double-blind trial of intravenous immunoglobulin for pemphigus. J Am Acad Dermatol. Apr 2009;60(4):595-603. [Medline].

  39. Toth GG, Jonkman MF. Successful treatment of recalcitrant penicillamine-induced pemphigus foliaceus by low-dose intravenous immunoglobulins. Br J Dermatol. Sep 1999;141(3):583-5. [Medline].

  40. Marzano AV, Fanoni D, Venegoni L, Berti E, Caputo R. Treatment of refractory pemphigus with the anti-CD20 monoclonal antibody (rituximab). Dermatology. 2007;214(4):310-8. [Medline].

  41. Alter M, Wittmann M, Volker B, Kapp A, Werfel T, Gutzmer R. [Successful treatment of pemphigus foliaceus with rituximab : Report of 3 cases.]. Hautarzt. Jan 18 2009;[Medline].

  42. Amagai M, Hashimoto T, Green KJ, Shimizu N, Nishikawa T. Antigen-specific immunoadsorption of pathogenic autoantibodies in pemphigus foliaceus. J Invest Dermatol. Jun 1995;104(6):895-901. [Medline].

  43. Baroni A, Perfetto B, Ruocco E, Greco R, Criscuolo D, Ruocco V. Cytokine pattern in blister fluid and sera of patients with pemphigus. Dermatology. 2002;205(2):116-21. [Medline].

  44. Beutner EH, Jordon RE, Chorzelski TP. The immunopathology of pemphigus and bullous pemphigoid. J Invest Dermatol. Aug 1968;51(2):63-80. [Medline].

  45. Brenner S, Bialy-Golan A, Anhalt GJ. Recognition of pemphigus antigens in drug-induced pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol. Jun 1997;36(6 Pt 1):919-23. [Medline].

  46. Bystryn JC, Steinman NM. The adjuvant therapy of pemphigus. An update. Arch Dermatol. Feb 1996;132(2):203-12. [Medline].

  47. Chernyavsky AI, Arredondo J, Piser T, Karlsson E, Grando SA. Differential coupling of M1 muscarinic and alpha7 nicotinic receptors to inhibition of pemphigus acantholysis. J Biol Chem. Feb 8 2008;283(6):3401-8. [Medline].

  48. Chorzelski TP, Von Weiss JF, Lever WF. Clinical significance of autoantibodies in pemphigus. Arch Dermatol. May 1966;93(5):570-6. [Medline].

  49. Dmochowski M. Are antibodies to desmosomal cadherins in pemphigus pathogenic?. Postepy Dermatol (Poznan). 1995;12:153-163.

  50. Dmochowski M, Nie Z, Kiyokawa C, Hashimoto T. Human desmocollin 1a transiently expressed in COS-7 cells and NIH 3T3-3 cells is reacted by IgG4 antibodies in a pemphigus foliaceus serum. J Dermatol Sci. Sep 1999;21(1):42-8. [Medline].

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

  52. Gomi H, Kawada A, Amagai M, Matsuo I. Pemphigus erythematosus: detection of anti-desmoglein-1 antibodies by ELISA. Dermatology. 1999;199(2):188-9. [Medline].

  53. Grando SA. Autoimmunity to keratinocyte acetylcholine receptors in pemphigus. Dermatology. 2000;201(4):290-5. [Medline].

  54. Grando SA. Biological functions of keratinocyte cholinergic receptors. J Investig Dermatol Symp Proc. Aug 1997;2(1):41-8. [Medline].

  55. Grando SA, Dahl MV. Nicotine and pemphigus. Arch Dermatol. Oct 2000;136(10):1269. [Medline].

  56. Grando SA, Grando AA, Glukhenky BT, Doguzov V, Nguyen VT, Holubar K. History and clinical significance of mechanical symptoms in blistering dermatoses: a reappraisal. J Am Acad Dermatol. Jan 2003;48(1):86-92. [Medline].

  57. Grando SA, Pittelkow MR, Shultz LD, Dmochowski M, Nguyen VT. Pemphigus: an unfolding story. J Invest Dermatol. Oct 2001;117(4):990-5. [Medline].

  58. Hameed A, Khan AA. Microscopic Nikolsky's sign. Clin Exp Dermatol. Jul 1999;24(4):312-4. [Medline].

  59. Harman KE, Gratian MJ, Bhogal BS, Challacombe SJ, Black MM. The use of two substrates to improve the sensitivity of indirect immunofluorescence in the diagnosis of pemphigus. Br J Dermatol. Jun 2000;142(6):1135-9. [Medline].

  60. Hernandez C, Amagai M, Chan LS. Pemphigus foliaceus: preferential binding of IgG1 and C3 at the upper epidermis. Br J Dermatol. Feb 1997;136(2):249-52. [Medline].

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

  62. Ishii K, Amagai M, Ohata Y, et al. Development of pemphigus vulgaris in a patient with pemphigus foliaceus: antidesmoglein antibody profile shift confirmed by enzyme-linked immunosorbent assay. J Am Acad Dermatol. May 2000;42(5 Pt 2):859-61. [Medline].

  63. Jakubowicz K, Sulej J, Chorzelski T. [Pemphigus herpetiformis: a new variant of pemphigus]. Przegl Dermatol. Sep-Dec 1981;68(5-6):583-7. [Medline].

  64. Joly P, Mokhtar I, Gilbert D, et al. Immunoblot and immunoelectronmicroscopic analysis of endemic Tunisian pemphigus. Br J Dermatol. Jan 1999;140(1):44-9. [Medline].

  65. Kano Y, Shimosegawa M, Mizukawa Y, Shiohara T. Pemphigus foliaceus induced by exposure to sunlight. Report of a case and analysis of photochallenge-induced lesions. Dermatology. 2000;201(2):132-8. [Medline].

  66. Kawana S, Hashimoto T, Nishikawa T, Nishiyama S. Changes in clinical features, histologic findings, and antigen profiles with development of pemphigus foliaceus from pemphigus vulgaris. Arch Dermatol. Dec 1994;130(12):1534-8. [Medline].

  67. Kozlowska A, Hashimoto T, Jarzabek-Chorzelska M, et al. Pemphigus herpetiformis with IgA and IgG antibodies to desmoglein 1 and IgG antibodies to desmocollin 3. J Am Acad Dermatol. Jan 2003;48(1):117-22. [Medline].

  68. Loiseau P, Lecleach L, Prost C, et al. HLA class II polymorphism contributes to specify desmoglein derived peptides in pemphigus vulgaris and pemphigus foliaceus. J Autoimmun. Aug 2000;15(1):67-73. [Medline].

  69. Lombardi ML, Mercuro O, Ruocco V, et al. Common human leukocyte antigen alleles in pemphigus vulgaris and pemphigus foliaceus Italian patients. J Invest Dermatol. Jul 1999;113(1):107-10. [Medline].

  70. Maciejowska E, Jablonska S, Chorzelski T. Is pemphigus herpetiformis an entity?. Int J Dermatol. Nov 1987;26(9):571-7. [Medline].

  71. Malik M, El Tal AE, Ahmed AR. Anal involvement in pemphigus vulgaris. Dis Colon Rectum. Apr 2006;49(4):500-6. [Medline].

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

  73. Musialowicz D, Hashimoto T, Jarzabek-Chorzelska M. Paraneoplastic pemphigus associated with spindle cell sarcoma. Coexistence of autoantibodies against plakins and desmogleins. Przegl Dermatol. 2000;87:139-146.

  74. Nguyen VT, Ndoye A, Bassler KD, et al. Classification, clinical manifestations, and immunopathological mechanisms of the epithelial variant of paraneoplastic autoimmune multiorgan syndrome: a reappraisal of paraneoplastic pemphigus. Arch Dermatol. Feb 2001;137(2):193-206. [Medline].

  75. Nguyen VT, Ndoye A, Shultz LD, Pittelkow MR, Grando SA. Antibodies against keratinocyte antigens other than desmogleins 1 and 3 can induce pemphigus vulgaris-like lesions. J Clin Invest. Dec 2000;106(12):1467-79. [Medline].

  76. Okusa Y, Tanaka M. Pemphigus foliaceus successfully treated with glycosporine. Acta Dermatol Kyoto. 1995;90:225-229.

  77. Ota M, Sato-Matsumura KC, Matsumura T, Tsuji Y, Ohkawara A. Pemphigus foliaceus and figurate erythema in a patient with prostate cancer. Br J Dermatol. Apr 2000;142(4):816-8. [Medline].

  78. Ozog DM, Gogstetter DS, Scott G, Gaspari AA. Minocycline-induced hyperpigmentation in patients with pemphigus and pemphigoid. Arch Dermatol. Sep 2000;136(9):1133-8. [Medline].

  79. Perry HO, Brunsting LA. Pemphigus foliaceus. Further observations. Arch Dermatol. Jan 1965;91:10-23. [Medline].

  80. Polifka M, Krusinski PA. The Nikolsky sign. Cutis. Nov 1980;26(5):521-5, 526. [Medline].

  81. Reis VM, Toledo RP, Lopez A, Diaz LA, Martins JE. UVB-induced acantholysis in endemic Pemphigus foliaceus (Fogo selvagem) and Pemphigus vulgaris. J Am Acad Dermatol. Apr 2000;42(4):571-6. [Medline].

  82. Robinson ND, Hashimoto T, Amagai M, Chan LS. The new pemphigus variants. J Am Acad Dermatol. May 1999;40(5 Pt 1):649-71; quiz 672-3. [Medline].

  83. Santi CG, Maruta CW, Aoki V, Sotto MN, Rivitti EA, Diaz LA. Pemphigus herpetiformis is a rare clinical expression of nonendemic pemphigus foliaceus, fogo selvagem, and pemphigus vulgaris. Cooperative Group on Fogo Selvagem Research. J Am Acad Dermatol. Jan 1996;34(1):40-6. [Medline].

  84. Seitz CS, Staegemeir E, Amagai M, Rose C, Brocker EB, Zillikens D. Pemphigus herpetiformis with an autoimmune response to recombinant desmoglein 1. Br J Dermatol. Aug 1999;141(2):354-5. [Medline].

  85. Shah N, Green AR, Elgart GW, Kerdel F. The use of chlorambucil with prednisone in the treatment of pemphigus. J Am Acad Dermatol. Jan 2000;42(1 Pt 1):85-8. [Medline].

  86. Tabolli S, Mozzetta A, Antinone V, Alfani S, Cianchini G, Abeni D. The health impact of pemphigus vulgaris and pemphigus foliaceus assessed using the Medical Outcomes Study 36-item short form health survey questionnaire. Br J Dermatol. Feb 22 2008;[Medline].

  87. Takashima H, Ohtsuyama M, Saito A. A case of pemphigus foliaceus with secondary erythroderma. Acta Dermatol Kyoto. 1996;91:363-367.

  88. Torrelo A, Hashimoto T, Mediero IG, Colmenero I, Zambrano A. Pemphigus foliaceous in a child. Clin Exp Dermatol. Mar 2006;31(2):288-9. [Medline].

  89. Torzecka JD, Sysa-Jedrzejowska A, Dziankowska-Bartkowiak B, Waszczykowska E. Pemphigus foliaceus: an unusual clinical feature and case reports. Cutis. Jan 1998;61(1):21-4. [Medline].

  90. Vieira JP. Penfigo foliaceo e syndromo de Senear-Uscher. Empresa Grafica da. 1942;Sao Paolo, Brazil.

  91. Vu TN, Lee TX, Ndoye A, et al. The pathophysiological significance of nondesmoglein targets of pemphigus autoimmunity. Development of antibodies against keratinocyte cholinergic receptors in patients with pemphigus vulgaris and pemphigus foliaceus. Arch Dermatol. Aug 1998;134(8):971-80. [Medline].

  92. Warren SJ, Arteaga LA, Rivitti EA, et al. The role of subclass switching in the pathogenesis of endemic pemphigus foliaceus. J Invest Dermatol. Jan 2003;120(1):104-8. [Medline].

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

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

superficial pemphigus, fogo selvagem, PF, pemphigus erythematosus, PE, pemphigus herpetiformis, PH, endemic PF, endemic pemphigus foliaceus, immunoglobulin A PF, IgA PF, IgA pemphigus foliaceus, immunoglobulin A pemphigus foliaceus, drug-induced PF, drug-induced pemphigus foliaceus

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Slawomir Majewski, MD, Professor and Director, Department of Dermatology and Venereology, Warsaw School of Medicine, Poland
Disclosure: Nothing to disclose.

Sebastian S Majewski, MD, Consulting Staff, Department of Dermatology, Military Institute of Health Services, Warsaw, Poland
Disclosure: Nothing to disclose.

Medical Editor

Robin Travers, MD, Assistant Professor of Medicine (Dermatology), Dartmouth University School of Medicine; Staff Dermatologist, New England Baptist Hospital; Private Practice, SkinCare Physicians
Robin Travers, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Informatics Association, Massachusetts Medical Society, Medical Dermatology Society, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Julia R Nunley, MD, Professor, Program Director, Dermatology Residency, Department of Dermatology, Virginia Commonwealth University Medical Center
Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, and Women's Dermatologic Society
Disclosure: Johnson and Johnson stock holder dividends; Amgen stock holder dividends; Forest Lab, Inc stock holder dividends; Galaxo Smith Klein stock holder dividends; Covidien stock holder dividends; Novartis Grant/research funds Consulting; Biolex  sub-investigator

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.