Acne Fulminans 

  • Author: Ryszard Zaba, MD, PhD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Mar 29, 2011
 

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.

Go to Acne Conglobata, Acne Keloidalis Nuchae, Acne Vulgaris, and Acneiform Eruptions for complete information on these topics.

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Pathophysiology

Acne fulminans is 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]

Acne fulminans has also been observed in patients with measles infection.[9]

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Epidemiology

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.

International

Approximately 100 patients with acne fulminans have been described.[10]

Age

Acne fulminans predominantly affects young males aged 13 to 22 years with a history of acne.

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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

Ryszard Zaba, MD, PhD is a member of the following medical societies: European Academy of Dermatology and Venereology and Sigma Xi

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, University of Medicine and Dentistry of New Jersey-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.

Specialty Editor Board

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

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.

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, Northside 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.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at 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.

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 Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.

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. Honma M, Murakami M, Iinuma S, et al. Acne fulminans following measles infection. J Dermatol. Aug 2009;36(8):471-3. [Medline].

  10. Zaba R, Schwartz R, Jarmuda S, Czarnecka-Operacz M, Silny W. Acne fulminans: explosive systemic form of acne. J Eur Acad Dermatol Venereol. Oct 3 2010;[Medline].

  11. 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].

  12. 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].

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

  14. 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].

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

  16. 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].

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

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

 
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