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Acne Fulminans

  • Author: Ryszard Zaba, MD, PhD; Chief Editor: William D James, MD  more...
 
Updated: Jun 06, 2016
 

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. Acne fulminans could be induced by anabolic steroid use in a male bodybuilder.[4] The trigger for acne induction seemed to be a testosterone therapy in a patient with Marfan syndrome.[5] . 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.[6] In addition to testosterone, isotretinoin may also precipitate acne fulminans, possibly related to highly increased levels of P acnes antigens in the patient'simmunesystem.[7, 8]

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.[9]

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.[10]

Acne fulminans has also been observed in patients with measles infection.[11]

Chronic inflammation in acne fulminans, characteristic to acne lesions, can also be accompanied by angiogenesis. By specific immunostaining for CD34+ endothelial cells, research revealed the presence of blood capillaries around the pilosebaceous follicles, within the inflammatory or pericystic infiltrate.[12]

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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.[13]

Age

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

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Prognosis

Recurrent AF is extremely rare. Bone lesions typically resolve with treatment, but residual radiographic changes, such as sclerosis and hyperostosis, may remain. Scarring and fibrosis may result from this acute inflammatory process.

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Patient Education

For patient education resources, see the Skin, Hair, and Nails Center and Acne.

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Contributor Information and Disclosures
Author

Ryszard Zaba, MD, PhD 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: Sigma Xi, European Academy of Dermatology and Venereology

Disclosure: Nothing to disclose.

Coauthor(s)

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 Meffert, MD Associate 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, Texas Dermatological Society

Disclosure: Nothing to disclose.

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

Joshua A Zeichner, MD Assistant Professor, Director of Cosmetic and Clinical Research, Mount Sinai School of Medicine; Chief of Dermatology, Institute for Family Health at North General

Joshua A Zeichner, MD is a member of the following medical societies: American Academy of Dermatology, National Psoriasis Foundation

Disclosure: Received consulting fee from Valeant for consulting; Received grant/research funds from Medicis for other; Received consulting fee from Galderma for consulting; Received consulting fee from Promius for consulting; Received consulting fee from Pharmaderm for consulting; Received consulting fee from Onset for consulting.

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. 1961 Dec. 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. 2005 May. 52(5 Suppl 1):S118-20. [Medline].

  4. Kraus SL, Emmert S, Schön MP, Haenssle HA. The dark side of beauty: acne fulminans induced by anabolic steroids in a male bodybuilder. Arch Dermatol. Oct 2012. 148(10):1210-2.

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

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

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

  8. Sarifakioglu E, Onur O, Kart H, Yilmaz AE. Acute myopathy and acne fulminans triggered by isotretinoin therapy. Eur J Dermatol. Sep-Oct 2011. 21(5):794-5. [Medline].

  9. 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].

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

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

  12. Brănişteanu DE, Cotrutz CE, Luca MC, Molodoi DA, Stoica LE, Ianoşi SL, et al. Morphopathological stigmata in acne fulminans. Rom J Morphol Embryol. 2015. 56 (3):1185-90. [Medline].

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

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

  15. Kim SY, Jung SK, Lee SG, Yi SM, Kim JH, Kim IH. Acne fulminans with osteolytic change in metaphysis of distal radius. Int J Dermatol. 2016 May. 55 (5):571-3. [Medline].

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

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

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

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

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

  21. Wakabayashi M, Fujimoto N, Uenishi T, Danno K, Tanaka T. A case of acne fulminans in a patient with ulcerative colitis successfully treated with prednisolone and diaminodiphenylsulfone: a literature review of acne fulminans, rosacea fulminans and neutrophilic dermatoses occurring in the setting of inflammatory bowel disease. Dermatology. 2011. 222(3):231-5. [Medline].

  22. Tago O, Nagai Y, Matsushima Y, Ishikawa O. A case of acne fulminans successfully treated with cyclosporin a and prednisolone. Acta Derm Venereol. May 2011. 91(3):337-8.

  23. Lages RB, Bona SH, Silva FV, Gomes AK, Campelo V. Acne fulminans successfully treated with prednisone and dapsone. An Bras Dermatol. Jul-Aug 2012. 87(4:612-4. [Medline].

  24. [Guideline] Zaenglein AL, Pathy AL, Schlosser BJ, Alikhan A, Baldwin HE, Berson DS, et al. Guidelines of care for the management of acne vulgaris. J Am Acad Dermatol. 2016 May. 74 (5):945-973.e33. [Medline].

  25. [Guideline] American Academy of Dermatology. Ten Things Physicians and Patients Should Question. Choosing Wisely. Available at http://www.choosingwisely.org/societies/american-academy-of-dermatology/. August 19, 2015; Accessed: : April 30, 2016.

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

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

 
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