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Pemphigus Foliaceus Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 13, 2016
 

History

The bullae usually start on the trunk. The course of the disease is long-term, with the patient's general health being satisfactory. Spontaneous remission sometimes occurs, but the lesions can persist for several years. A unique clinical pattern may occur in children, with individual lesions appearing as arcuate, circinate, or polycyclic.[27] Eyelid skin involvement without conjunctival changes occurs occasionally in patients with pemphigus foliaceus.[28]

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Physical

The primary lesions are small, superficial blisters; however, these flaccid bullae are difficult to find because they are transient and transform into erosions. Typical pemphigus foliaceus has scaly, crusted erosions on an erythematosus base confined mainly to so-called seborrhoic areas (eg, face, scalp, upper part of the trunk).

The Nikolsky sign is the finding that physical trauma can shear the pathologic epidermis of the skin of patients with pemphigus foliaceus, resulting in clinical lesions. The Nikolsky sign should probably be regarded as a moderately sensitive but highly specific tool for the diagnosis of pemphigus.[29]

The erosions can become numerous, showing a tendency to generalize. Occasionally, erythrodermia develops. Thus, pemphigus foliaceus may be evident as a an erythroderma or a psoriasiform erythroderma.[26, 30] Atrophic changes of the nails and the hair are sometimes evident. The erosions may be accompanied by a burning sensation and local pain. In contrast to PV, in pemphigus foliaceus, little or no involvement of the mucous membranes occurs. Note the images below.

Middle-aged American woman of Mexican lineage with Middle-aged American woman of Mexican lineage with superficial bullae characteristic of pemphigus foliaceus.
Pemphigus foliaceus. Middle-aged American woman of Pemphigus foliaceus. Middle-aged American woman of Mexican lineage with superficial bullae formation.
A 41-year-old woman of Puerto Rican origin with a A 41-year-old woman of Puerto Rican origin with a 9-year history of pemphigus foliaceus, often with erythroderma flares.
A 41-year-old woman of Puerto Rican origin with a A 41-year-old woman of Puerto Rican origin with a 9-year history of pemphigus foliaceus, often with erythroderma flares.

IgA pemphigus foliaceus begins as pruritic, flaccid vesicles in an annular pattern.

PH commences as intensely pruritic, grouped papules and vesicles suggestive of dermatitis herpetiformis. Erythematous patches with peripheral vesicles may be present. Sometimes, oral erosions are seen.

PE starts as erythematous patches with border vesiculation, often in a butterfly distribution on the cheeks and the forehead, with similar patches on the sternal and interscapular skin. Crusted plaques may appear in the healing phase.

PNP is a subset of pemphigus combining the clinical features of PV variably associated with those of erythema multiforme, bullous pemphigoid, and lichen planus. Chorzelski and associates[7] in 1999 described a most unusual case of PNP with the immunopathologic findings of pemphigus foliaceus. The clinical pattern appears to be correlated with that of the antibody profile; therefore, patients with antibodies directed against desmoglein 1 tend to have the clinical features of pemphigus foliaceus.

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Causes

Endemic pemphigus foliaceus, or fogo selvagem, seems to be induced by a viral infection transmitted by insects.

In some patients, pemphigus foliaceus may be precipitated by extensive UV exposure or burns and by various drugs (eg, penicillamine,[31, 32] inhibitors of angiotensin convertase, nonsteroid anti-inflammatory agents).[33] Pemphigus foliaceus induced by bucillamine has been described.[34]

A topical ectoparasiticide containing fipronil, amitraz, and S-methoprene has been described as causing pemphigus foliaceus in dogs.[35]

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Contributor Information and Disclosures
Author

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.

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 

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

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, Women's Dermatologic Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Board of Dermatology<br/>Co-Editor for the text Dermatological Manifestations of Kidney Disease .

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

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, Women's Dermatologic Society, Medical Dermatology Society

Disclosure: Nothing to disclose.

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. 1968 Apr. 80(4):211-7. [Medline].

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

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

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

  6. 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. 2003 Oct. 49(4):609-14. [Medline].

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

  8. Grando SA, Holubar K, Schwartz RA. Jean-Claude Bystryn 1938-2010. An obituary. Exp Dermatol. 2011 Mar. 20(3):293-6. [Medline].

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

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

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

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

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

  14. Kikuchi K, Inokuma D, Moriuchi R, Koguchi-Yoshioka H, Yasui C, Shimizu S. Exacerbation of Pemphigus Foliaceus After Electron-beam Radiation. Acta Derm Venereol. 2014 Mar 13. [Medline].

  15. Inadomi T. A case of pemphigus foliaceus aggravated in an irradiated area by radiotherapy against breast cancer. Indian J Dermatol. 2015 Jan-Feb. 60(1):93-4. [Medline]. [Full Text].

  16. Liebman TN, Lieberman MR, Burris K. Pemphigus foliaceus exacerbated by radiation, in association with myasthenia gravis. Dermatol Online J. 2016 Mar 16. 22 (3):360-4. [Medline].

  17. Brochado MJ, Nascimento DF, Campos W, Deghaide NH, Donadi EA, Roselino AM. Differential HLA class I and class II associations in pemphigus foliaceus and pemphigus vulgaris patients from a prevalent Southeastern Brazilian region. J Autoimmun. 2016 May 10. [Medline].

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

  19. Ruocco V. Attualita' sul pemfigo indotto. 2003.

  20. 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. 2008 Feb. 22(2):242-4. [Medline].

  21. Dalla-Costa R, Pincerati MR, Beltrame MH, Malheiros D, Petzl-Erler ML. Polymorphisms In the 2q33 and 3q21 Chromosome Regions Including T Cell Coreceptor and Ligand Genes May Influence Susceptibility to Pemphigus Foliaceus. Hum Immunol. 2010 Apr 27. [Medline].

  22. Shelly S, Agmon-Levin N, Altman A, Shoenfeld Y. Thymoma and autoimmunity. Cell Mol Immunol. 2011 Feb 14. [Medline].

  23. Toumi A, Saleh MA, Yamagami J, Abida O, Kallel M, Masmoudi A, et al. Autoimmune reactivity against precursor form of desmoglein 1 in healthy Tunisians in the area of endemic pemphigus foliaceus. J Dermatol Sci. 2013 Feb 19. [Medline].

  24. 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].

  25. 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. 2000 Jul 6. 343(1):23-30. [Medline].

  26. Fariba G, Ayatollahi A, Hejazi S. Pemphigus foliaceus. Indian Pediatr. 2012 Mar 8. 49(3):240-1. [Medline].

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

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

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

  30. Grekin SJ, Fox MC, Gudjonsson JE, Fullen DR. Psoriasiform pemphigus foliaceus: a report of two cases. J Cutan Pathol. 2012 Jan 10. [Medline].

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

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

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

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

  35. Bizikova P, Linder KE, Olivry T. Fipronil-amitraz-S-methoprene-triggered pemphigus foliaceus in 21 dogs: clinical, histological and immunological characteristics. Vet Dermatol. 2014 Apr. 25(2):103-e30. [Medline].

  36. 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. 2008 Jan. 47(1):61-3. [Medline].

  37. 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].

  38. Kumaresan M, Rai R, Sandhya V. Immunofluorescence of the outer root sheath: an aid to diagnosis in pemphigus. Clin Exp Dermatol. 2010 Dec 24. [Medline].

  39. 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. 2000 May. 25(3):236-40. [Medline].

  40. 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. 2000 Apr. 142(4):812-3. [Medline].

  41. 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.

  42. 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. 2005 Jan. 30(1):43-5. [Medline].

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

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

  45. 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. 2008 Jun. 58(6):1043-6. [Medline].

  46. 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. 1999 Jun. 140(6):1127-9. [Medline].

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

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

  49. 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. 1993 Jun. 28(6):998-1000. [Medline].

  50. 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. 2005 Feb. 35(4):427-8. [Medline].

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

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

  53. Gee SN, Velez NF, Sepehr A, Burgin S. Two distinct viral infections complicating pemphigus foliaceus. Dermatol Online J. 2012 Jan 15. 18(1):3. [Medline].

  54. Martin LK, Werth VP, Villaneuva EV, Murrell DF. A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol. 2011 Feb 24. [Medline].

  55. Daniel BS, Murrell DF. The actual management of pemphigus. G Ital Dermatol Venereol. 2010 Oct. 145(5):689-702. [Medline].

  56. 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].

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

  58. Lolis M, Toosi S, Czernik A, Bystryn JC. Effect of intravenous immunoglobulin with or without cytotoxic drugs on pemphigus intercellular antibodies. J Am Acad Dermatol. 2011 Mar. 64(3):484-9. [Medline].

  59. Iskandarli M, Gerceker Turk B, Ertam I, Yaman B, Ozturk G. Successful treatment of Wilson disease-associated IgA pemphigus with IVIG. J Eur Acad Dermatol Venereol. 2015 Mar 12. [Medline].

  60. Doukaki S, Platamone A, Alaimo R, Bongiorno MR. Mycophenolate mofetil and enteric-coated mycophenolate sodium in the treatment of pemphigus vulgaris and pemphigus foliaceus. J Dermatolog Treat. 2014 Feb 13. [Medline].

  61. de Sena Nogueira Maehara L, Huizinga J, Jonkman MF. Rituximab therapy in pemphigus foliaceus: report of 12 cases and review of recent literature. Br J Dermatol. 2014 Dec 13. [Medline].

 
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Middle-aged American woman of Mexican lineage with superficial bullae characteristic of pemphigus foliaceus.
Pemphigus foliaceus. Middle-aged American woman of Mexican lineage with superficial bullae formation.
A 41-year-old woman of Puerto Rican origin with a 9-year history of pemphigus foliaceus, often with erythroderma flares.
A 41-year-old woman of Puerto Rican origin with a 9-year history of pemphigus foliaceus, often with erythroderma flares.
Histologic view shows the typical pattern of a detached stratum corneum without bullae formation. Pigmentary incontinence is prominent in the dermis, reflecting the patient's 9-year history of recurrent superficial bullae.
 
 
 
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