Acne Conglobata 

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Apr 25, 2011
 

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. KuflikNodules on the back. Courtesy of Emanuel G. Kuflik. Nodules on the face. Courtesy of Emanuel G. KuflikNodules on the face. Courtesy of Emanuel G. Kuflik. Nodules and pustules on the back. Courtesy of EmanNodules and pustules on the back. Courtesy of Emanuel G. Kuflik. A close-up view of nodules and pustules on the bacA 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.

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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.

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Epidemiology

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.

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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, 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.

Coauthor(s)

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.

Specialty Editor Board

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.

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.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M 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.

References

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