eMedicine Specialties > Dermatology > Diseases of the Adnexa

Acne Fulminans

Author: Ryszard Zaba, MD, PhD, Director, Department of Dermatology, Professor, Department of Dermatology and Venereology, Poznan University School of Medical Sciences, Poland
Coauthor(s): 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
Contributor Information and Disclosures

Updated: Mar 12, 2009

Introduction

Background

Acne fulminans (AF), also known as acne maligna, was originally described as acute febrile ulcerative acne conglobata (AC). In 1958, at a meeting of the Detroit Dermatological Society, Burns and Colville presented a 16-year-old white boy with acute febrile disease and acne conglobata. Many similar cases have been reported since then.1 The primary features of this disease include sudden onset, severe and often ulcerating acne, fever, polyarthritis,2 and failure to respond to antibacterial therapy; the response to debridement in combination with steroid therapy is good. It can be the dermatologic manifestation of the synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome.3 Acne fulminans is a syndrome of fulminant, necrotizing acne associated with bone lesions, constitutional symptoms, and laboratory abnormalities.

Other acne-related eMedicine articles include Acne Conglobata, Acne Keloidalis Nuchae, Acne Vulgaris, and Acneiform Eruptions.

Pathophysiology

Acne fulminansis an uncommon, immunologically induced, systemic disease in which the triggering antigen is believed to be from Propionibacterium acnes. Some authors note that elevated blood levels of testosterone may play an important role in the pathogenesis of acne fulminans. High levels of testosterone and anabolic steroids cause an increase in sebum excretion and in the population density of P acnes. The trigger for acne induction seemed to be a testosterone therapy in a patient with Marfan syndrome.4 The increase in the amount of P acnes or related antigens may trigger the immunologic reaction in some individuals and lead to an occurrence of acne fulminans.5 In addition to testosterone, isotretinoin may also precipitate acne fulminans, possibly related to highly increased levels of P acnes antigens in the patient's immune system.6

Another theory postulates that acne fulminans may be an autoimmune complex disease because circulating immune complexes have been demonstrated in some patients with acne fulminans. Immunologically, the reaction is a type III or IV hypersensitivity reaction.

Genetic factors may play an important role in some patients; 4 sets of identical twins who developed an identical pattern of acne fulminans have been documented.7

Acne may be the only clinical sign of androgen excess in men, and one report is available about a boy with acne fulminans and androgen excess due to late-onset congenital adrenal hyperplasia.8

Frequency

United States

Acne fulminans is a rare disease. Over the past several years, fewer cases of this disease have occurred, possibly because of earlier and better treatment of acne.

Age

Acne fulminans predominantly affects young males with a history of acne.

Clinical

History

  • The primary features of this disease include the following:
    • Sudden onset
    • Severe and often ulcerating acne
    • Fever and polyarthritis
    • Failure to respond to antibacterial therapy
    • Good response to oral steroid therapy, after 4-6 weeks, the addition of oral isotretinoin
  • Acne fulminans predominantly affects young males with a history of acne.
  • Painful splenomegaly and erythema nodosum may be present.9
  • Bone pain related to aseptic osteolysis may be present. Gordon et al report a case of a 13-year-old boy with severe acne and multiple osteolytic bone lesions who presented to pediatric oncologists; the patient avoided unnecessary painful diagnostic procedures when it was recognized he had acne fulminans.10
  • Patients with acne fulminans and acneiform folliculitis may have chronic aseptic multifocal osteomyelitis.

Physical

  • Acne fulminans (similar to acne conglobata) demonstrates numerous inflammatory nodules on the trunk. In acne fulminans, the large nodules tend to become painful ulcers with overhanging borders surrounding exudative necrotic plaques that become confluent; however, polyporous comedones and noninflammatory cysts are not evident (as seen in acne conglobata). Erythematous neovascular nodules may also be seen.
  • Acne fulminans is a systemic disease. Patients may demonstrate a bent-over posture because polyarthritis may make walking painful.
  • Inflammatory arthralgia may affect 1 joint or several joints, especially the hips, the knees, and the thighs.

Causes

See Pathophysiology.

More on Acne Fulminans

Overview: Acne Fulminans
Differential Diagnoses & Workup: Acne Fulminans
Treatment & Medication: Acne Fulminans
Follow-up: Acne Fulminans
References
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References

  1. Burns RE, Colville JM. Acne conglobata with septicemia. Arch Dermatol. 1959;79:361-3.

  2. Windom RE, Sanford JP, Ziff M. Acne conglobata and arthritis. Arthritis Rheum. Dec 1961;4:632-5. [Medline].

  3. Iqbal M, Kolodney MS. Acne fulminans with synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome treated with infliximab. J Am Acad Dermatol. May 2005;52(5 Suppl 1):S118-20. [Medline].

  4. Wollina U, Gesina H, Koch A, Kostler E. Case reports: acne fulminans in Marfan syndrome. J Drugs Dermatol. Jul-Aug 2005;4(4):501-5. [Medline].

  5. Heydenreich G. Testosterone and anabolic steroids and acne fulminans. Arch Dermatol. Apr 1989;125(4):571-2. [Medline].

  6. Kalbarczyk K, Ciupinska M. Complications during treatment with Roaccutane, acne fulminans 2001. Dermatol Klin (Wroclaw). 2001;3 (Suppl 1):130.

  7. Palatsi R, Oikarinen A. Hormonal analysis and delayed hypersensitivity reactions in identical twins with severe acne. Acta Derm Venereol. 1979;59(2):157-60. [Medline].

  8. Placzek M, Degitz K, Schmidt H, Plewig G. Acne fulminans in late-onset congenital adrenal hyperplasia. Lancet. Aug 28 1999;354(9180):739-40. [Medline].

  9. Williamson DM, Cunliffe WJ, Gatecliff M, Scott DG. Acute ulcerative acne conglobata (acne fulminans) with erythema nodosum. Clin Exp Dermatol. Dec 1977;2(4):351-4. [Medline].

  10. Gordon PM, Farr PM, Milligan A. Acne fulminans and bone lesions may present to other specialties. Pediatr Dermatol. Nov-Dec 1997;14(6):446-8. [Medline].

  11. Allison MA, Dunn CL, Person DA. Acne fulminans treated with isotretinoin and "pulse" corticosteroids. Pediatr Dermatol. Jan-Feb 1997;14(1):39-42. [Medline].

  12. Leyden JJ. The role of isotretinoin in the treatment of acne: personal observations. J Am Acad Dermatol. Aug 1998;39(2 Pt 3):S45-9. [Medline].

  13. Seukeran DC, Cunliffe WJ. The treatment of acne fulminans: a review of 25 cases. Br J Dermatol. Aug 1999;141(2):307-9. [Medline].

  14. Friedlander SF. Effective treatment of acne fulminans-associated granulation tissue with the pulsed dye laser. Pediatr Dermatol. Sep-Oct 1998;15(5):396-8. [Medline].

  15. Champion RH, Burton JL, Burns DA, Breathnach SM, eds. Rook/Wilkinson/Ebling Textbook of Dermatology. 6th ed. Oxford, England: Blackwell Science; 1998:1967-79.

  16. Cunliffe WJ, Gollnick H. Acne fulminans. In: Cunliffe WJ, Gollnick H. Acne. Diagnosis and Management. London, England: Martin Dunitz Ltd; 2001:84-6.

  17. Jansen T, Plewig G. Acne fulminans. Int J Dermatol. Apr 1998;37(4):254-7. [Medline].

  18. Jansen T, Romiti R, Plewig G. Acute severe acne in a female patient (acne fulminans?). Br J Dermatol. Nov 1999;141(5):945-7. [Medline].

  19. Thomson KF, Cunliffe WJ. Acne fulminans 'sine fulminans'. Clin Exp Dermatol. Jun 2000;25(4):299-301. [Medline].

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Further Reading

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Keywords

acne fulminans, acute febrile ulcerative conglobata acne with polyarthritis, acute febrile ulcerative conglobata acne with leukemoid reaction, acne maligna, acute ulcerative acne conglobata

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Contributor Information and Disclosures

Author

Ryszard Zaba, MD, PhD, Director, Department of Dermatology, Professor, Department of Dermatology and Venereology, Poznan University School of Medical Sciences, Poland
Disclosure: Nothing to disclose.

Coauthor(s)

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.

Medical Editor

Alexa F Boer Kimball, MD, MPH, Associate Professor of Dermatology, Harvard University School of Medicine; Vice Chair, Department of Dermatology, Massachusetts General Hospital; Director of Clinical Unit for Research Trials in Skin (CURTIS), Department of Dermatology, Massachusetts General Hospital and Brigham and Women's Hospital
Alexa F Boer Kimball, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
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