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Acne Conglobata

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Last Update: December 2, 2018.

Introduction

Acne conglobata is a rare but severe form of acne. It usually presents with deep burrowing abscesses that interconnect with each other. Scar formation and disfigurement of the body are common with this type of acne. The comedones often occur in groups of three and the cysts often contain purulent, foul smelling material that is discharged on the skin surface. Acne conglobata nodules are found on the shoulder, chest, upper arms, buttocks, face, and thigh. Acne conglobata may occur following the sudden worsening of a pustular acne, or the disorder may occur gradually following recrudescence of acne that has been quiet for many years.[1][2][3]

It is important not to confuse acne conglobata with acne fulminans. They both present with inflammatory nodules on the trunk. The major difference is that the former induces polyporous comedones and non-inflammatory cysts, whereas the latter does not.

Etiology

It is believed that Propionibacterium acnes, the same organism implicated in acne vulgaris, may play an important role in the disease by changing its reactivity as an antigen. The hypersensitivity to this antigen induces an intense immunological reaction that presents with a chronic inflammatory state. The presence of the bacteria also leads to an infectious process with the development of pus and sinus tracts. The pus is usually foul smelling and putrid.[4][5][6]

Ingestion of thyroid medication and exposure to halogenated aromatic hydrocarbons may trigger acne conglobata. Other factors that can provoke acne conglobata include androgens (e.g., androgen-producing tumors), and anabolic steroids.

Acne conglobata has also been reported to occur in individuals who stop anabolic hormones or as a reaction to other hormonal agents.

Some people with acne conglobata have the XYY karyotype. The skin disorder has been linked to HLA phenotypes.

Epidemiology

Acne conglobata is not very common. Overall, it is more common in men than women. Over the past 30 years, many reports have been published on athletes with this type of acne, and this is felt to be primarily to the use of anabolic steroids. Outside the US, not much is known about acne conglobata. The condition is usually seen in young adults and very rarely seen in children or elderly people. The onset is usually in the second and third decade of life. Whether it occurs more frequently in any specific race is not known.[7]

Pathophysiology

Acne conglobata presents with deep burrowing abscesses that interconnect with each other via sinus tracts. Initially, the nodular lesion may mimic a pimple, but underneath there is a vigorous inflammatory reaction and formation of pus. Over time, the pus pushes into the adjacent tissues and extrudes on the skin surface. Scar formation and disfigurement of the body are common with this type of acne. The comedones often occur in groups of three, and the cysts often contain purulent, foul-smelling material that is discharged on the skin surface.

Histopathology

Acne Conglobata resembles hidradenitis suppurativa with large tender nodules and draining sinus tracts. The involved area is intensely inflamed and fluctuant. Foreign body granulomas are common.

History and Physical

Acne conglobata may follow the use of androgenic anabolic steroids and is quite common in bodybuilders. Many young adult males will present to the dermatology clinic complaining of severe acne and facial scars. It is important to seek a thorough history of use of anabolic steroids because discontinuation of these agents is vital for treatment.

Acne conglobata may also occur in patients with hidradenitis suppurativa and pyoderma gangrenosum. When the condition develops following puberty, the nodules will gradually coalesce and increase in severity over the ensuing years. Active nodule formation usually persists for the first three decades of life and then becomes quiescent.

A physical exam will usually reveal a severe form of acne. The nodular lesions are tender and dome-shaped. When the nodules have broken down, there will be the presence of discharge that is foul smelling pus. After the pus has drained, crusting of the lesion is common, followed by formation of large irregular scars.

A classic feature of the disorder is the presence of paired or aggregates of blackheads on the trunk, neck, upper arms, and buttocks.

The majority of patients with acne conglobata are shy and embarrassed about the skin condition. Many give a history of being withdrawn and isolated. Suicidal ideations are also common in this population. Thus, it is vital to offer them some mental health counseling.

Evaluation

The diagnosis is made clinically, and the discharge should be cultured. Appropriate antibiotics should be started in the presence of putrid discharge. One should not wait for culture results before starting antibiotic therapy.

Treatment / Management

The treatment of choice for acne conglobata is with the use of retinoids like isotretinoin for 20 to 28 weeks or in some cases even longer. Some experts even recommend the use of oral prednisone (1 mg/kg/d) for 14 to 28 days. Steroids have been shown to be effective when there are systemic constitutional symptoms such as general malaise, fever, weight loss, and anorexia. Topical retinoids are not as effective as oral retinoids. It is important not to administer retinoids to women of childbearing age in the absence of effective contraception as these drugs are known to be teratogenic.[8][9]

  • Other options include the use of minocycline, tetracycline, or doxycycline. The tetracyclines should not be combined with oral isotretinoin because there is a real potential to induce pseudotumor cerebri.
  • In cases which are unresponsive to the above antibiotics, dapsone is an option. There are also case reports of treatment of acne conglobata with infliximab and carbon dioxide laser with or without isotretinoin.
  • In severe cases of acne conglobata which do not respond to the above treatments, another option is external beam radiation.

Surgery

  • Once the lesions have healed, dermal fillers can be used to improve the scars. Recently the FDA approved the use of the bovine collagen filler, Bellafill, for treatment of acne scarring.
  • When nodules are large and fluctuant, they can be aspirated. Sometimes practitioners may use cryotherapy or intralesional triamcinolone. The large nodules can also be excised surgically.

Enhancing Healthcare Team Outcomes

Patients with acne usually present to their primary care provider or nurse practitioner. But these healthcare workers should be aware that there are types of acne which are very serious and need an appropriate consult with a dermatologist. Acne conglobata is best managed by a skin physician as it requires more aggressive therapy with close follow up.

In patients with acne conglobata, a significant disfigurement is common, and the scarring often results in psychological impairment and isolation from society. Many people with acne conglobata develop depression and anxiety. Once the diagnosis is made, these individuals should receive psychological counseling. The tendency is for these patients to hide the body disfigurement and skin lesions with garments which often leads to excess warmth and humidity, which worsens the skin condition. Hence, a practitioner should educate the patient on skin hygiene and recommend counseling. Starting these patients on antidepressants and anti-anxiety medications can be helpful.[10] (Level V)

Questions

To access free multiple choice questions on this topic, click here.

References

1.
Al-Kathiri L, Al-Najjar T. Severe Nodulocystic Acne not Responding to Isotretinoin Therapy Successfully Treated with Oral Dapsone. Oman Med J. 2018 Sep;33(5):433-436. [PMC free article: PMC6131924] [PubMed: 30210724]
2.
Borgia F, Vaccaro M, Giuffrida R, Cannavò SP. Photodynamic therapy for acne conglobata of the buttocks: Effective antiinflammatory treatment with good cosmetic outcome. Indian J Dermatol Venereol Leprol. 2018 Sep-Oct;84(5):617-619. [PubMed: 30073983]
3.
Sotoodian B, Kuzel P, Brassard A, Fiorillo L. Disfiguring ulcerative neutrophilic dermatosis secondary to doxycycline and isotretinoin in an adolescent boy with acne conglobata. Cutis. 2017 Dec;100(6):E23-E26. [PubMed: 29360905]
4.
Balakirski G, Neis MM, Megahed M. Acne conglobata induced by adalimumab. Eur J Dermatol. 2017 Jun 01;27(3):320-321. [PubMed: 28524046]
5.
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 07; [PubMed: 28480529]
6.
Dessinioti C, Katsambas A. Difficult and rare forms of acne. Clin. Dermatol. 2017 Mar - Apr;35(2):138-146. [PubMed: 28274350]
7.
Scheinfeld N. Diseases associated with hidranitis suppurativa: part 2 of a series on hidradenitis. Dermatol. Online J. 2013 Jun 15;19(6):18558. [PubMed: 24011308]
8.
Yiu ZZ, Madan V, Griffiths CE. Acne conglobata and adalimumab: use of tumour necrosis factor-α antagonists in treatment-resistant acne conglobata, and review of the literature. Clin. Exp. Dermatol. 2015 Jun;40(4):383-6. [PubMed: 25545016]
9.
Sand FL, Thomsen SF. Adalimumab for the treatment of refractory acne conglobata. JAMA Dermatol. 2013 Nov;149(11):1306-7. [PubMed: 24048280]
10.
Harth W, Hillert A, Hermes B, Seikowski K, Niemeier V, Freudenmann RW. [Suicidal behavior in dermatology]. Hautarzt. 2008 Apr;59(4):289-96. [PubMed: 18338146]
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Bookshelf ID: NBK459219PMID: 29083736

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