Acne Conglobata

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Acne Conglobata

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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. See the images below.

Pyogenic arthritis, pyoderma gangrenosum, and acne conglobata are clinically distinct inflammatory disorders that may be seen rarely in the same patient in a syndrome known as PAPA Syndrome. It was originally reported in a 3-generation kindred with autosomal dominant transmission The PAPA syndrome is related to the triad of pyoderma gangrenosum, acne conglobata, and suppurative hidradenitis, known as the PASH syndrome. [2] The simultaneous presence of pyoderma gangrenosum, acne conglobata, suppurative hidradenitis, and seronegative spondyloarthritis has been suggested as a new linkage with the designation being PASS syndrome. [3] Micali and Lacarrubba adroitly observed that secondary comedones in acne conglobata may correlate with double-ended pseudocomedones in hidradenitis suppurativa. [4]

Acne conglobata may also be associated with the SAPHO syndrome, which consists of synovitis, acne conglobata, pustulosis, hyperostosis, and osteitis. SAPHO syndrome is characterized by distinctive osteoarticular manifestations and a spectrum of neutrophilic dermatoses, including palmoplantar pustulosis. [5] It should be considered in patients with osteoarticular pain, particularly involving the anterior chest wall and/or spine, and neutrophilic skin lesions.

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

The primary causes of acne conglobata remain unknown. Chromosomal defects in the XYY 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. [6]

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. [7]

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). [8] 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.

The primary cause of acne conglobata remains unknown.

Changes in reactivity to Cutibacterium acnes (formerly 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. [9]

Acne conglobata is an uncommon disease.

The disease affects males more frequently than females.

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

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.

For patient education resources, see the Skin, Hair, and Nails Center and Teen Health Center, as well as Acne and Abscess.

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

Braun-Falco M, Kovnerystyy O, Lohse P, Ruzicka T. Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH)-a new autoinflammatory syndrome distinct from PAPA syndrome. J Am Acad Dermatol. 2012 Mar. 66(3):409-15. [Medline].

Bruzzese V. Pyoderma gangrenosum, acne conglobata, suppurative hidradenitis, and axial spondyloarthritis: efficacy of anti-tumor necrosis factor a therapy. J Clin Rheumatol. 2012 Dec. 18(8):413-5. [Medline].

Lacarrubba M, Micali G. Secondary Comedones in a Case of Acne Conglobata Correlate with Double-ended Pseudocomedones in Hidradenitis Suppurativa. Acta Derm Venereol. 2017 Mar 30. [Medline].

Zuo RC, Schwartz DM, Lee CC, Anadkat MJ, Cowen EW, Naik HB. Palmoplantar pustules and osteoarticular pain in a 42-year-old woman. J Am Acad Dermatol. 2015 Mar. 72(3):550-3. [Medline]. [Full Text].

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

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. 1974 Sep. 110(3):468. [Medline].

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

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

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

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

Pagano A, Feldman MJ. Management of chronic gluteal follicular occlusive disease. Eplasty. 2013. 13:ic50. [Medline]. [Full Text].

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

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

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. 1984 Oct-Dec. 2(4):309-11. [Medline].

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

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. 2001 Apr. 39(2):141-4. [Medline].

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

Lim DT, James NM, Hassan S, Khan MA. Spondyloarthritis associated with acne conglobata, hidradenitis suppurativa and dissecting cellulitis of the scalp: a review with illustrative cases. Curr Rheumatol Rep. 2013 Aug. 15(8):346. [Medline].

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

Freira S, Fonseca H, Ferreira PD, Vasconcelos P, Fonseca JE. SAPHO syndrome in an adolescent: a clinical case with unusual severe systemic impact. J Adolesc Health. 2014 Aug. 55(2):304-6. [Medline].

Leone A, Cassar-Pullicino VN, Casale R, Magarelli N, Semprini A, Colosimo C. The SAPHO syndrome revisited with an emphasis on spinal manifestations. Skeletal Radiol. 2015 Jan. 44(1):9-24. [Medline].

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. 2001 Aug. 145(2):363-4. [Medline].

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

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

Inoue CN, Tanaka Y, Tabata N. Acne conglobata in a long-term survivor with trisomy 13, accompanied by selective IgM deficiency. Am J Med Genet A. 2017 May 7. [Medline].

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

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

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

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. 2007 Aug. 34(8):583-5. [Medline].

Myers JN, Mason AR, Gillespie LK, Salkey KS. Treatment of acne conglobata with modern external beam radiation. J Am Acad Dermatol. 2009 Aug 6. [Medline].

Wolber C, David-Jelinek K, Udvardi A, Artacker G, Volc-Platzer B, Kurz H. [Successful therapy of sacroiliitis in SAPHO syndrome by etanercept.]. Wien Med Wochenschr. 2011 Jan 25. [Medline].

Garcovich S, Amelia R, Magarelli N, Valenza V, Amerio P. Long-term treatment of severe SAPHO syndrome with adalimumab: case report and a review of the literature. Am J Clin Dermatol. 2012 Feb 1. 13(1):55-9. [Medline].

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

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

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

Misery L, Feton-Danou N, Consoli A, Chastaing M, Consoli S, Schollhammer M. [Isotretinoin and adolescent depression]. Ann Dermatol Venereol. 2012 Feb. 139(2):118-23. [Medline].

[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].

[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.

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

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. 2009 Feb. 36(2):118-9. [Medline].

Yiu ZZ, Madan V, Griffiths CE. Acne conglobata and adalimumab: use of tumour necrosis factor-a antagonists in treatment-resistant acne conglobata, and review of the literature. Clin Exp Dermatol. 2014 Dec 26. [Medline].

Balakirski G, Neis MM, Megahed M. Acne conglobata induced by adalimumab. Eur J Dermatol. 2017 May 19. [Medline].

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.

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.

David F Butler, MD Former 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: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery, Association of Military Dermatologists, Phi Beta Kappa

Disclosure: Nothing to disclose.

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

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.

Acne Conglobata

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Acne Conglobata
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