Acne Conglobata 

Updated: Apr 24, 2019
Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD 

Overview

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. See the images below.

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

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.

Pathophysiology

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.

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]

Epidemiology

Frequency

Acne conglobata is an uncommon disease.

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.

Prognosis

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.

Patient Education

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

 

Presentation

History

Both acne conglobata and acne fulminans (AF) can be induced by anabolic-androgenic steroid abuse.[10] 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.[11] The hidradenitis may extensively involve the perineal and gluteal regions.[12]

The list of possible associations of pyoderma gangrenosum must include acne conglobata.[13]

The association of acne conglobata and arthritis is rare, and has been reported only in single case reports in the literature,[14, 15, 16] although musculoskeletal syndrome (ie, myalgia, arthralgia, arthritis, hyperostosis) developed in some patients with severe acne (acne conglobata and acne fulminans).[17]

Pyoderma gangrenosum, acne conglobata, and immunoglobulin A gammopathy has been observed.[18]

Spondyloarthritis associated with acne conglobata, hidradenitis suppurativa, and dissecting cellulitis of the scalp has been reported.[19]

Renal amyloidosis may accompany acne conglobata.[20]

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.

The SAPHO syndrome of synovitis, acne conglobata, pustulosis, hyperostosis, and osteitis is rare, but should not be overlooked.[5, 21, 22] Affected individuals may have difficulty walking, owing to pain, weakness, and weight loss.

Physical Examination

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. 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. See the image below.

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

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. See the image below.

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.[23]

Complications

This inflammatory form of acne may be associated with necrotizing scleritis, a potentially sight‐threatening subtype of anterior scleritis sometimes seen with underlying inflammatory disorders.[24]

 

DDx

Diagnostic Considerations

Halogenoderma must be considered, especially iododerma and bromoderma. Similarly, the chlorinated chemical dioxin in high doses (eg, when used as a poison) can produce acneiform papulonodules resembling acne fulminans.[25]

Acne conglobata (AC) resembles acne fulminans because both cause numerous inflammatory nodules on the trunk. Acne conglobata produces polyporous comedones and noninflammatory cysts, while acne fulminans does not. Unlike acne conglobata, large nodules of acne fulminans tend to become painful ulcers with overhanging borders surrounding exudative necrotic plaques that become confluent.

Mycobacterium chelonae I infection has been described as a mimic of acne conglobata in an immunocompetent host.[26]

The SAPHO syndrome, of which acne conglobata may be a prominent component, may have spinal imaging findings that require distinction from infection and cancer.[22]

Acne conglobata has been described with trisomy 13 and selective IgM deficiency.[27]

Differential Diagnoses

 

Treatment

Medical Care

The therapy of choice for acne conglobata (AC) includes isotretinoin 0.5-1 mg/kg for 4-6 months.

Simultaneous use of systemic steroids, such as prednisone 1 mg/kg/d for 2-4 weeks, may also prove beneficial, particularly if systemic symptoms are evident.

Alternatives include oral tetracycline, minocycline, or doxycycline. Oral tetracycline antibiotics should not be combined with oral isotretinoin because of an increased risk of pseudotumor cerebri.

For treatment-resistant cases, dapsone 50-150 mg/d is recommended; this treatment should be carefully monitored.[28]

Along with vigorous medical therapy, emotional support is essential.

Treatment of acne conglobata with infliximab has been reported.[29]

Successful treatment of perifolliculitis capitis abscedens et suffodiens, including acne conglobata, has been described with combined isotretinoin and dapsone.[30]

Acne conglobata has been successfully treated by carbon dioxide laser combined with topical tretinoin therapy.[31]

When severe acne conglobata is unresponsive to more accepted options, modern external beam radiation may be an alternative.[32]

The triad of pyoderma gangrenosum, acne, and suppurative hidradenitis, the so-called PASH syndrome, may respond to interleukin 1-beta blockade.[2]

Patients with SAPHO syndrome may benefit from the use of etanercept.[33] or combination treatment with isotretinoin and adalimumab, an antitumor necrosis factor-alpha monoclonal antibody.[34] Another option may be infliximab infusion.[3]

In December 2014, the US Food and Drug Administration (FDA) approved Bellafill, the first dermal filler indicated for acne scarring. Bellafill is a bovine collagen dermal filler.

Surgical Care

Large hemorrhagic nodules may be aspirated.

Intralesional triamcinolone or cryotherapy may also be valuable.[35]

Occasionally, surgical excision of interconnecting large nodules may be beneficial.[36]

Consultations

Failure to recognize and treat acne conglobata (AC) can produce considerable disfigurement. Suicidal ideation, a concern in seemingly healthy adolescents, should be anticipated in those with cosmetically disturbing skin disorders, especially in adolescent males with acne conglobata.[37] It may be wise to order routine screening for psychological disturbance in adolescents with acne conglobata. Use of isotretinoin is not contraindicated in those with depression.[38]

 

Guidelines

Guidelines Summary

In 2016, the American Academy of Dermatology (AAD) issued new evidence-based guidelines for acne vulgaris treatment of both adolescents and adults. Recommended treatments include topical therapy, antibiotics, isotretinoin, and oral contraceptives.[39] The key recommendations include the following:

  • Benzoyl peroxide or combinations with erythromycin or clindamycin as monotherapy for mild acne; benzoyl peroxide with a topical retinoid or systemic antibiotic therapy for moderate-to-severe acne

  • Topical antibiotics (eg, erythromycin, clindamycin) are not recommended as monotherapy because of the risk of bacterial resistance

  • Topical retinoids as monotherapy in primarily comedonal acne, or in combination with topical or oral antimicrobials for mixed or primarily inflammatory acne

  • Topical adapalene, tretinoin, and benzoyl peroxide can be safely used to treat acne in preadolescent children

  • Topical dapsone 5% gel for inflammatory acne, particularly in adult females

  • Systemic antibiotics are recommended for moderate and severe acne and forms of inflammatory acne that are resistant to topical treatments; doxycycline and minocycline are both more effective than tetracycline

  • Topical therapy with benzoyl peroxide or a retinoid should be used with systemic antibiotics and for maintenance after completion of systemic antibiotic therapy

  • Monotherapy with systemic antibiotics is not recommended

  • Systemic antibiotic use should be limited to the shortest possible duration; to minimize the development of bacterial resistance, reevaluation at 3-4 months

  • Use of oral erythromycin and azithromycin should be limited to those who cannot use the tetracyclines (ie, pregnant women or children aged < 8 y); erythromycin use should be restricted because of its increased risk of bacterial resistance

  • Isotretinoin is recommended for severe acne or moderate acne that does not respond to other therapy; low-dose isotretinoin can be used to effectively treat acne and reduce the frequency and severity of medication-related adverse effects, but intermittent dosing is not recommended; all patients treated with isotretinoin must adhere to the iPLEDGE risk management program; patients should receive routine monitoring of liver function tests, serum cholesterol, and triglycerides at baseline and again until response to treatment is established, but routine monitoring of complete blood count is not recommended; patients should be educated about the potential risks and monitored for any indication of inflammatory bowel disease and depressive symptoms

  • Combined oral contraceptives (COC) containing estrogen are effective for treatment of inflammatory acne in females; physicians should follow the World Health Organization (WHO) recommendations for COC usage eligibility

  • Despite the lack of published data, relying on available evidence, experience, and expert opinion, the guidelines support the use of spironolactone in select women

In 2015, as part of the Choosing Wisely® initiative from the American Board of Internal Medicine Foundation (ABIM), the AAD released recommendations regarding low-value care that cautioned against the routine use of microbiologic testing in the evaluation and management of acne. The AAD concluded that determining the type of bacteria present in acne lesions was unnecessary because it did not alter the management of typical acne presentations.[40]

 

Medication

Medication Summary

The goals of pharmacotherapy and surgical approaches are to reduce morbidity and to prevent complications. They may be combined.[41] For example, acne conglobata can be treated by fractional laser after carbon dioxide laser abrasion of cysts combined with topical tretinoin therapy.[42] The tumor necrosis factor-alpha antagonist adalimumab may represent another option for acne conglobata resistant to conventional therapies.[43, 44]

Corticosteroids

Class Summary

Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

Prednisone

Prednisone is a synthetic adrenocortical steroid with predominantly glucocorticoid properties. It ddecreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reducing capillary permeability. It stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.

Prednisolone (Orapred, Pediapred, Millipred)

Corticosteroids act as potent inhibitors of inflammation. They may cause profound and varied metabolic effects, particularly in relation to salt, water, and glucose tolerance, in addition to their modification of the immune response of the body.

Retinoid-like Agents, Topical

Class Summary

Vitamin A derivatives stimulate cellular retinoid receptors and help normalize keratinocyte differentiation and are comedolytic. In addition, they have anti-inflammatory properties. Oral isotretinoin also reduces sebum production in the skin.

Isotretinoin (Claravis, Amnesteem, Sotret)

Isotretinoin is an oral agent that treats serious dermatologic conditions. Isotretinoin is the synthetic 13-cis isomer of the naturally occurring tretinoin (trans -retinoic acid). Both agents are structurally related to beta-carotene. It decreases sebaceous gland size and sebum production. It may inhibit sebaceous gland differentiation and abnormal keratinization. Effective March 1, 2006, the FDA requires that prescribers of isotretinoin, patients who take isotretinoin, and pharmacists who dispense isotretinoin all must register with the iPLEDGE system.

Tretinoin topical (Avita, Retin-A, Retin-A Micro, Tretin-X)

Tretinoin topical is structurally related to vitamin A. It may be helpful for recalcitrant disease, but recurrence is common. Long-term, low-dose therapy may be suitable for selected patients. It may cause skin irritation in some patients. Also, it has been linked to the promotion of angiogenesis; however, it has not demonstrated increased telangiectasias. Tretinoin topical also inhibits microcomedo formation and eliminates lesions. It makes keratinocytes in sebaceous follicles less adherent and easier to remove. It is available as creams and gels.

Antibiotics, Other

Class Summary

Therapy must be comprehensive and cover all likely pathogens in the context of the clinical setting. Antibiotic selection should be guided by blood culture sensitivity whenever feasible.

Doxycyline (Doryx, Adoxa, Vibramycin, Doxy 100)

Doxycyline treats infections caused by susceptible gram-negative and gram-positive organisms. It inhibits protein synthesis and, thus, bacterial growth by binding to 30S and possibly 50S ribosomal subunits of susceptible bacteria. It may block dissociation of peptidyl t-RNA from ribosomes, causing RNA-dependent protein synthesis to arrest. The brand name 150-mg Doryx is enteric coated, to reduce gastrointestinal adverse effects.

Minocycline (Solodyn, Minocin, Dynacin)

Minocycline treats infections caused by susceptible gram-negative and gram-positive organisms, in addition to infections caused by susceptible Chlamydia, Rickettsia, and Mycoplasma species. The brand name Solodyn is an extended-release formulation indicated for acne and prescribed as a weight based 1-mg/kg dose per day. The minocycline dose should be lowered in patients with renal impairment.

Tetracycline

Tetracycline inhibits bacterial protein synthesis by binding with 30S and, possibly, 50S ribosomal subunit(s). Tetracycline has anti-inflammatory activity. One may administer 250-500 mg orally twice daily.

 

Questions & Answers

Overview

What is acne conglobata (AC)?

What is the relationship between acne conglobata (AC) and PAPA syndrome?

What is the relationship between acne conglobata (AC) and SAPHO syndrome?

What is the pathophysiology of acne conglobata (AC)?

What causes acne conglobata (AC)?

What is the prevalence of acne conglobata (AC)?

What are the sexual predilections of acne conglobata (AC)?

At what age does acne conglobata (AC) typically present?

What is the prognosis of acne conglobata (AC)?

Presentation

Which clinical history findings are characteristic of acne conglobata (AC)?

Which physical findings are characteristic of acne conglobata (AC)?

What are the possible complications of acne conglobata (AC)?

DDX

Which conditions are included in the differential diagnoses of acne conglobata (AC)?

What are the differential diagnoses for Acne Conglobata?

Treatment

How is acne conglobata (AC) treated?

What is the role of surgery in the treatment of acne conglobata (AC)?

Which specialist consultations are beneficial to patients with acne conglobata (AC)?

Guidelines

What are the AAD guidelines on the treatment of acne conglobata (AC)?

Medications

What is the role of medications in the treatment of acne conglobata (AC)?

Which medications in the drug class Antibiotics, Other are used in the treatment of Acne Conglobata?

Which medications in the drug class Retinoid-like Agents, Topical are used in the treatment of Acne Conglobata?

Which medications in the drug class Corticosteroids are used in the treatment of Acne Conglobata?