eMedicine Specialties > Dermatology > Diseases of the Adnexa

Acne Conglobata

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

Updated: Jun 4, 2009

Introduction

Background

Acne conglobata (AC) is an uncommon and unusually severe form of acne characterized by burrowing and interconnecting abscesses and irregular scars (both keloidal and atrophic), often producing pronounced disfigurement. The comedones often occur in a group of 2 or 3, and cysts contain foul-smelling seropurulent material that returns after drainage. The nodules are usually found on the chest, the shoulders, the back, the buttocks, the upper arms, the thighs, and the face.1 Acne conglobata may develop as a result of a sudden deterioration of existing active papular or pustular acne, or it may occur as the recrudescence of acne that has been quiescent for many years.

Nodules on the back. Courtesy of Emanuel G. Kufli...

Nodules on the back. Courtesy of Emanuel G. Kuflik.

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Nodules on the back. Courtesy of Emanuel G. Kufli...

Nodules on the back. Courtesy of Emanuel G. Kuflik.



Nodules on the face. Courtesy of Emanuel G. Kufli...

Nodules on the face. Courtesy of Emanuel G. Kuflik.

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Nodules on the face. Courtesy of Emanuel G. Kufli...

Nodules on the face. Courtesy of Emanuel G. Kuflik.



Nodules and pustules on the back. Courtesy of Ema...

Nodules and pustules on the back. Courtesy of Emanuel G. Kuflik.

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Nodules and pustules on the back. Courtesy of Ema...

Nodules and pustules on the back. Courtesy of Emanuel G. Kuflik.



A close-up view of nodules and pustules on the ba...

A close-up view of nodules and pustules on the back. Courtesy of Emanuel G. Kuflik.

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A close-up view of nodules and pustules on the ba...

A close-up view of nodules and pustules on the back. Courtesy of Emanuel G. Kuflik.

Pyoderma gangrenosum, acne conglobata, and aseptic arthritis are clinically distinct inflammatory disorders. Although this triad of symptoms rarely occurs in an individual patient, it was reported in a 3-generation kindred with autosomal dominant transmission of the 3 disorders; this condition is called familial pyoderma gangrenosum, acne conglobata, and aseptic arthritis (PAPA) syndrome.

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

Pathophysiology

The primary causes of acne conglobata remain unknown. Chromosomal defects in the XXY karyotype may be responsible for severe forms of acne conglobata. In contrast, the XXY karyotype of Klinefelter syndrome is believed to exclude severe acne; however, 1 patient with the unusual combination of Klinefelter syndrome and acne conglobata has been reported.2

The association of this disease with specific human leukocyte antigen (HLA) phenotypes has not been proven. The HLA-A and HLA-B phenotypes were evaluated in 65 patients with acne conglobata, in whom antigen frequencies were found to be normal. Other patients with acne conglobata and hidradenitis suppurativa were studied; 4 of 6 patients had HLA-B7 cross-reacting antigens (ie, HLA-B7, HLA-Bw22, HLA-B27, HLA-Bw40, HLA-Bw42), and all had HLA-DRw4.3

PAPA syndrome has been mapped to a locus on the long arm of chromosome 15 (maximum 2-point logarithm of odds score 5.83; recombination fraction [straight theta] 0 at locus D15S206).4 Assuming complete penetrance, haplotype analysis of recombination events defined an interval of 10 centimorgans between loci D15S1023 and D15S979. This finding suggests that these clinically distinct disorders may share a genetic etiology.

Frequency

International

Acne conglobata is an uncommon disease.

Mortality/Morbidity

Acne conglobata can produce pronounced disfigurement. Severe scarring produces psychological impairment; individuals with acne conglobata are often ostracized, or they may feel excluded. Acne conglobata has also been responsible for anxiety and depression in many patients.

Sex

The disease affects males more frequently than females.

Age

The onset of acne conglobata usually occurs in young adults aged 18-30 years, but infants may develop this condition as well.

Clinical

History

Both acne conglobata and acne fulminans (AF) can be induced by anabolic-androgenic steroid abuse.5 Although this probably represents only a small minority of cases, one should recognize bodybuilding acne, address the substance abuse, and warn patients about other potential hazards.

  • Acne conglobata can be associated with hidradenitis suppurativa. Note that hidradenitis suppurativa occurs more frequently in patients with mild acne than in other patients.6
  • The list of possible associations of pyoderma gangrenosum must include acne conglobata.7
  • The association of acne conglobata and arthritis is rare, and it has been reported only in single case reports in the literature,8,9,10 although musculoskeletal syndrome (ie, myalgia, arthralgia, arthritis, hyperostosis) developed in some patients with severe acne (acne conglobata and acne fulminans).11
  • Pyoderma gangrenosum, acne conglobata, and immunoglobulin A gammopathy has been observed.12
  • Renal amyloidosis may accompany acne conglobata.13
  • Acne conglobata and hidradenitis suppurativa may have a familial tendency; however, no significant relationship in the antigen patterns of patients with acne conglobata was observed.

Physical

  • The draining sinus is a malevolent lesion usually seen in severe forms of acne, such as acne conglobata, acne fulminans, and acne inversa.
  • In patients with acne conglobata and sacroiliitis, acute anterior uveitis may occur.
  • The nodules associated with acne conglobata are succulent, tender, and dome shaped.
    • Characteristic nodules increase in size; break down to discharge pus; and often fuse, forming unusual shapes of several centimeters.

      A close-up view of nodules and pustules on the fo...

      A close-up view of nodules and pustules on the forehead. Courtesy of Emanuel G. Kuflik.

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      A close-up view of nodules and pustules on the fo...

      A close-up view of nodules and pustules on the forehead. Courtesy of Emanuel G. Kuflik.

    • The formation of nodules begins in early puberty; the severity increases until late adolescence and often beyond. Active nodule formation may persist for years and usually continues until the fourth decade of life.
  • Isolation of coagulase-positive staphylococci is common in the lesions.
  • As the nodules break down, crusts may form over a deep ulcer, which extends centrifugally but tends to heal centrally. This process is persistent, and slow healing is characteristic.


A closer view of nodules and pustules on the back...

A closer view of nodules and pustules on the back. Courtesy of Emanuel G. Kuflik.

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A closer view of nodules and pustules on the back...

A closer view of nodules and pustules on the back. Courtesy of Emanuel G. Kuflik.

  • A conspicuous feature of the disease is the blackheads that appear in pairs or groups on the neck or the trunk; sometimes, blackheads involve the upper arms or the buttocks.14

Causes

  • The primary cause of acne conglobata remains unknown.
  • Changes in reactivity to Propionibacterium acnes may play an important role in the etiology of the disease.
  • Exposure to halogenated aromatic hydrocarbons (eg, dioxins) or ingestion of halogens (eg, thyroid medication, hypnotic agents) may trigger acne conglobata in an individual who is predisposed.
  • Other factors that can provoke acne conglobata include androgens (eg, androgen-producing tumors) and anabolic steroids.
  • Acne conglobata and acne fulminans may appear after cessation of testosterone therapy or as a reaction to other medications.15

More on Acne Conglobata

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

  1. Patterson WM, Stibich AS, Dobke M, Schwartz RA. Mutilating facial acne conglobata. Cutis. Aug 2000;66(2):139-40. [Medline].

  2. Wollenberg A, Wolff H, Jansen T, Schmid MH, Rocken M, Plewig G. Acne conglobata and Klinefelter's syndrome. Br J Dermatol. Mar 1997;136(3):421-3. [Medline].

  3. Schackert K, Scholz S, Steinbauer-Rosenthal I, Albert ED, Wank R, Plewig G. Letter: HL-A antigens in acne conglobata: a negative study. Arch Dermatol. Sep 1974;110(3):468. [Medline].

  4. Yeon HB, Lindor NM, Seidman JG, Seidman CE. Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome maps to chromosome 15q. Am J Hum Genet. Apr 2000;66(4):1443-8. [Medline].

  5. Melnik B, Jansen T, Grabbe S. Abuse of anabolic-androgenic steroids and bodybuilding acne: an underestimated health problem. J Dtsch Dermatol Ges. Feb 2007;5(2):110-7. [Medline].

  6. Leybishkis B, Fasseas P, Ryan KF, Roy R. Hidradenitis suppurativa and acne conglobata associated with spondyloarthropathy. Am J Med Sci. Mar 2001;321(3):195-7. [Medline].

  7. Velez A, Alcala J, Fernandez-Roldan JC. Pyoderma gangrenosum associated with acne conglobata. Clin Exp Dermatol. Nov 1995;20(6):496-8. [Medline].

  8. Ehrenfeld M, Samra Y, Kaplinsky N. Acne conglobata and arthritis: report of a case and review of the literature. Clin Rheumatol. Sep 1986;5(3):407-9. [Medline].

  9. Vasey FB, Fenske NA, Clement GB, Bridgeford PH, Germain BF, Espinoza LR. Immunological studies of the arthritis of acne conglobata and hidradenitis suppurativa. Clin Exp Rheumatol. Oct-Dec 1984;2(4):309-11. [Medline].

  10. Ziff M, Maliakkal J. Acne conglobata and arthritis. Ann Intern Med. Mar 1983;98(3):417. [Medline].

  11. Roldán JC, Terheyden H, Dunsche A, Kampen WU, Schroeder JO. Acne with chronic recurrent multifocal osteomyelitis involving the mandible as part of the SAPHO syndrome: case report. Br J Oral Maxillofac Surg. Apr 2001;39(2):141-4. [Medline].

  12. Birnkrant MJ, Papadopoulos AJ, Schwartz RA, Lambert WC. Pyoderma gangrenosum, acne conglobata, and IgA gammopathy. Int J Dermatol. Mar 2003;42(3):213-6. [Medline].

  13. Perez-Villa F, Campistol JM, Ferrando J, Botey A. Renal amyloidosis secondary to acne conglobata. Int J Dermatol. Mar 1989;28(2):132-3. [Medline].

  14. Shimomura Y, Nomoto S, Yamada S, Ito A, Ito K, Ito M. Chronic glomerulonephritis remarkably improved after surgery for acne conglobata of the buttocks. Br J Dermatol. Aug 2001;145(2):363-4. [Medline].

  15. el-Shahawy MA, Gadallah MF, Massry SG. Acne: a potential side effect of cyclosporine A therapy. Nephron. 1996;72(4):679-82. [Medline].

  16. Sterling JB, Hanke CW. Dioxin toxicity and chloracne in the Ukraine. J Drugs Dermatol. Mar-Apr 2005;4(2):148-50. [Medline].

  17. Ena P, Zanetti S, Sechi LA. Mycobacterium chelonae I infection mimicking acne conglobata in an immunocompetent host. Clin Exp Dermatol. Jul 2004;29(4):423-5. [Medline].

  18. Tan BB, Lear JT, Smith AG. Acne fulminans and erythema nodosum during isotretinoin therapy responding to dapsone. Clin Exp Dermatol. Jan 1997;22(1):26-7. [Medline].

  19. Shirakawa M, Uramoto K, Harada FA. Treatment of acne conglobata with infliximab. J Am Acad Dermatol. Aug 2006;55(2):344-6. [Medline].

  20. Bolz S, Jappe U, Hartschuh W. Successful treatment of perifolliculitis capitis abscedens et suffodiens with combined isotretinoin and dapsone. J Dtsch Dermatol Ges. Jan 2008;6(1):44-7. [Medline].

  21. Hasegawa T, Matsukura T, Suga Y, et al. Case of acne conglobata successfully treated by CO(2) laser combined with topical tretinoin therapy. J Dermatol. Aug 2007;34(8):583-5. [Medline].

  22. Leyden JJ, Mills OH, Kligman AM. Cryoprobe treatment of acne conglobata. Br J Dermatol. Mar 1974;90(3):335-41. [Medline].

  23. Weinrauch L, Peled I, Hacham-Zadeh S, Wexler MR. Surgical treatment of severe acne conglobata. J Dermatol Surg Oncol. Jun 1981;7(6):492-4. [Medline].

  24. Jeong S, Lee CW. Acne conglobata: treatment with isotretinoin, colchicine, and cyclosporin as compared with surgical intervention. Clin Exp Dermatol. Nov 1996;21(6):462-3. [Medline].

  25. Hasegawa T, Matsukura T, Hirasawa Y, et al. Acne conglobata successfully treated by fractional laser after CO laser abrasion of cysts combined with topical tretinoin. J Dermatol. Feb 2009;36(2):118-9. [Medline].

  26. Harth W, Hillert A, Hermes B, Seikowski K, Niemeier V, Freudenmann RW. [Suicidal behavior in dermatology]. Hautarzt. Apr 2008;59(4):289-96. [Medline].

  27. Champion RH, Burton JL, Burns DA, Breathnach SM, eds. Rook/Wilkinson/Ebling Textbook of Dermatology. Oxford, England: Blackwell Science; 1998:1967, 1979.

  28. Cunliffe WJ. Acne. 3rd ed. London, England: Martin Dunitz Publishers; 1993.

  29. Jansen T, Burgdorf WH, Plewig G. Pathogenesis and treatment of acne in childhood. Pediatr Dermatol. Jan-Feb 1997;14(1):17-21. [Medline].

  30. Plewig G, Kligman AM. Acne and Rosacea. Berlin: Springer-Verlag; 1993:351-5.

  31. Zaba R. Pathogenesis and treatment of acne vulgaris. Postepy Dermatol Alergol (Postepy). 2001;18:131-140.

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Further Reading

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Keywords

acne conglobata, AC, pyoderma gangrenosum, aseptic arthritis, PAPA syndrome, acne fulminans, AF

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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)

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.

Medical Editor

Shyam Verma, MBBS, DVD, FAAD, Adjunct Clinical Assistant Professor, Department of Dermatology, University of Virginia, State University of New York at Stonybrook, Penn State University
Shyam Verma, MBBS, DVD, FAAD is a member of the following medical societies: American Academy of 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, 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.

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

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.

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