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What Is Acne Conglobata?

Acne Conglobata Is a Rare and Unusually Severe Form of Acne

Last updated: May 14, 2019

Article Summary

Acne conglobata is a severe form of acne that presents a range of lesions, most notably nodules, cysts, blackheads, abscesses, and draining sinuses. The disease typically affects young males, but females may develop it too. Acne conglobata is difficult to treat and it often leaves massive, disfiguring scars. The treatment of choice that is often prescribed in combination with oral corticosteroids is isotretinoin, often known by its brand name Accutane®.

Definition

Acne conglobata, a rare, severe form of acne that normally affects 18 - 30-year-old males, is very difficult to treat, and often leaves massive, disfiguring scars.1,2 This form of acne falls under the umbrella term nodulocystic acne, which is used to refer to any type of acne that features, but is not necessarily limited to, large, red, painful acne lesions called nodules and/or cysts. Acne conglobata also often comes with blackheads and lesions called draining sinuses, which are large lesions that comprise multiple cysts that connect to one another. 

Incidence

Acne conglobata is very uncommon. Typical patients are males between 18 and 30, though females can get it as well. In rare cases, infants may also suffer from the disease.1 For unknown reasons, the disease is more prevalent in tropical climates.3

Causes

Nobody knows what causes acne conglobata, but the research suggests that the following three factors play a role. 

  1. Genetics
  2. Elevated hormones
  3. Increased sensitivity to bacteria

Possible Causes of Acne Conglobata

Genetics

Researchers suspect there is a gene responsible for predisposition to acne conglobata because evidence shows that if a person's parents had acne conglobata, that person's chances of getting it are much higher. 

Elevated androgens

Medical professionals suspect that androgens (male sex hormones present in both males and females) contribute to the disease, particularly because androgens peak during puberty and adolescent males get affected most often.1 Increased dehydroepiandrosterone sulfate (DHEAS) and testosterone (both male sex hormones) are a frequent finding in patients with nodulocystic acne, but no major studies have been performed to determine if that is also true for acne conglobata. 

Increased sensitivity to bacteria

Some researchers put the blame on increased sensitivity to Propionibacterium acnes (P. acnes) (bacteria associated with acne). They hypothesize that the immune system in people with acne conglobata overreacts to the presence of the bacteria that, in other individuals, would bring about just regular acne.1

Symptoms

Acne conglobata can develop from existing acne or in individuals who have never had a problem with acne. Once the disease manifests, it usually persists for months or even years.2 Lesions begin to form in the late teen years, and the disease often persists into the late twenties and, sometimes, early thirties.

Features

Acne conglobata is an intense form of acne that affects large areas of the skin, including the chest, back, neck, face, shoulders, limbs, and buttocks, and very often leaves massive, disfiguring scars. Acne conglobata patients present with a combination of lesions of all kinds, most notably nodules, cysts, blackheads, abscesses, and draining sinuses (several cysts merged together under the skin).1,2Whiteheads, papules, and pustules may be present as well, but they are not a dominant feature.

  • Nodules and cysts: Nodules and cysts in acne conglobata are plentiful. Both types of acne lesions are over five millimeters in diameter and are tender and painful. The difference is that nodules are fibrous lesions that do not contain pus, whereas cysts contain "foul-smelling"pus-like material that returns after you drain them. Healing of both nodules and cysts can be extremely slow, often resulting in massive, disfiguring scars. Scars in acne conglobata can be indented or raised above the skin
  • Communicating comedones: Very typical of acne conglobata are lesions called communicating comedones, and can include double comedones and triple comedones, which are two to three blackheads that have merged into one extra-large blackhead. Communicating comedones commonly occur on the neck, the trunk, and, less frequently, on the upperarmsand buttocks.1
  • Abscesses: Abscesses are collections of pus that build up in the skin and tissue underneath the skin in response to harmful bacteria. They are red, painful, and feel fluid-filled when touched. Abscesses are surrounded by a wall of healthy cells, also called an abscess capsule, which the immune system builds to protect the body from the pus and bacteria inside the abscess.
  • Draining sinuses: Draining sinuses are large, inflammatory, elongated lesions that form when two or more cysts merge together under the skin. Draining sinuses are a serious complication of chronic (persisting for a long time) acne conglobata. From the surface, a draining sinus is a red, elevated bump that is two to five centimeters long and periodically discharges pus. They are most common on the face, particularly between the nose and upper lip, and also on the neck.
  • Scars: Scars in acne conglobata are often widespread and can be atrophic (indented) or hypertrophic (raised above the skin).

Acne Conglobata Lesion Types

Acne conglobata vs. other severe forms of acne 

One should be careful when differentiating acne conglobata from other severe forms of nodulocystic acne--acne fulminans and pyoderma faciale

Acne Conglobata Vs. Other Severe Forms of Acne

Acne fulminans: In acne fulminans, the lesions tend to appear on the same areas of the face and body as in acne conglobata. Also, like in acne conglobata, neighboring nodules/cysts often merge together and form extra-large inflammatory lesions in acne fulminans. What helps to differentiate between the two conditions is that nodules and cysts in acne fulminans fill with blood and rapidly turn into open wounds, while in acne conglobata they normally do not fill with blood and persist as raised lesions covered by the skin for long periods of time. In addition, acne fulminans commonly presents systemic symptoms (symptoms that affect the whole body), such as fever, joint pain, and weight loss, which are very uncommon in acne conglobata.5

Pyoderma faciale: Pyoderma faciale is another dramatic form of nodulocystic acne, characterized by a sudden emergence of intense redness and large merging nodules and/or cysts on the face. The disease is relatively easy to differentiate from acne conglobata because pyoderma faciale occurs exclusively in females and affects the face only.

Characteristics of Nodulocystic Acne Types

Treatment

Acne conglobata is difficult but possible to treat. Isotretinoin is normally the number one choice, with several other treatment options available.1 It is a highly controversial drug that must be used only under close supervision by a doctor and is associated with numerous hazardous health effects. Only the severity of the disease justifies the use of this treatment option. 

First-line treatments

First-line Treatment for Acne Conglobata Isotretinoin

  • Isotretinoin: An isotretinoin course off our to six months is the most common treatment option.1 The studies show that isotretinoin reduces the total number of lesions by 60 - 95%, on average, depending on the dose and duration of treatment.6-8

Clinical trials on the effectiveness of isotretinoin in acne conglobata

The New England Journal of Medicine

In a 1979 study published in The New England Journal of Medicine,14 patients with severe nodulocystic acne including, but not limited to, acne conglobata underwent a four-month treatment course with isotretinoin at a high dosage of 2mg/kg/day, which is more than usually prescribed these days.6 All but one patient, 75% of whose lesions cleared, experienced 100% clearance of the lesions. Most of the facial cysts/nodules cleared in the first month, while lesions on the trunk were more stubborn and took longer to resolve, particularly in males. Some degree of remission was achieved in all 14 patients. Twenty months after the completion of the treatment, 11 patients remained free of nodules/cysts, and the other 3 patients shared a total of nine cysts or nodules. The researchers did not specify how many cysts or nodules each person had, but we can say with assurance that they had drastically fewer than they had before treatment.

Der Hautartzt

A study published in the German journal Der Hautartzt in 1983 reported good results with isotretinoin in 171 patients with acne conglobata.7 The patients were allocated to three groups, receiving either 0.2, 0.5, or 1mg/kg/day of isotretinoin for 24 weeks. After 12 weeks, the researchers adjusted the dose based on how well acne cleared. If at least two thirds of lesions resolved, the initial dosage of 0.2mg/kg/day was continued for another 12 weeks; 0.5mg/kg/day was reduced to 0.2mg/kg/day, and 1mg/kg/day was either reduced to 0.2mg/kg/day or isotretinoin simply was discontinued. If less than two thirds of lesions cleared, 0.2mg/kg/day was raised to 0.5mg/kg/day and 0.5mg/kg/day was raised to 1mg/kg/day. The patients in the 1mg/kg/day group noticed the best results. Full results of the study are given in the table below.

Isotretinoin Study Results

Zeitschrift fur Hautkrankheiten

A study on 46 patients, published in another German journal Zeitschrift fur Hautkrankheiten in 1984, reported comparable results: after six months of therapy with isotretinoin, the overall number of lesions reduced, on average, by 94.4% on the face and 85.8% on the chest and back.8 The patients initially received 40, 60, or 80mg of isotretinoin daily, which, for an average 70-kilo person, would equate to approximately 0.5 - 1mg/kg/day, and the doses were then adjusted according to how well the patients responded to treatment.

Isotretinoin's effectiveness in acne conglobata treatment likely lies in its ability to reduce production of sebum (skin oil).9-11Sebum is a crucial factor in the development of acne. In fact, individuals who do not produce sebum, such as castrated men, never experience acne. 

Clinical trials on how isotretinoin reduces sebum in people with nodulocystic acne

Journal of the American Academy of Dermatology

In a 1980 study in the Journal of the American Academy of Dermatology, 1mg/kg/day of isotretinoin taken orally for 12 weeks reduced sebum production in patients with nodulocystic acne by almost 90%.9 A study published in the Journal of the American Academy of Dermatology in 1984 reported that 40mg of isotretinoin daily for 16 weeks decreased the production of sebum by up to 55%.10

Journal of Investigative Dermatology

A 1986 study in the Journal of Investigative Dermatology discovered that as sebum production dropped with the administration of isotretinoin, the amount of P. acnes in the affected skin pores reduced as well.11 This makes sense because P. acnes feeds on sebum. This finding is particularly relevant because the study was performed on 40 patients with acne conglobata.

Despite being highly effective for severe acne, isotretinoin often comes with a long list of side effects and, sometimes, even severe toxic reactions. This is why isotretinoin is approved for use only for severe, nodulocystic acne.

Side Effects

The most frequent side effects include:

  • Inflammation of lips (90%*) 
  • Dryness of skin and mucous membranes (80%) 
  • Pink eye (40%)
  • Itching (40%) 
  • Gastrointestinal symptoms, such as nausea, vomiting, and abdominal pain (20%) 
  • Muscle and/or joint pain (16%)
  • Increased sensitivity to light (10%)

    *% incidence

Toxic reactions: Possible toxic reactions associated with the administration of isotretinoin might include inflammatory bowel disease and increased intracranial (in the head) pressure that causes moderate to severe headaches and visual impairments, such as blurred and double vision, difficulty seeing to the side, and brief episodes of blindness.12 The incidence of these events is unknown, but they are rather uncommon. 

Adverse effect on fetus: EXTREME CAUTION! Isotretinoin is contraindicated in pregnancy due to its severe adverse effect on the fetus.12 In fact, isotretinoin is the leading birth defect - causing medication on the market. Birth defects are often very severe, including death. This is why it is strongly recommended for females to obtain two negative pregnancy tests prior to starting therapy. Additionally, women must use two forms of contraception for 30 days both before and after treatment with isotretinoin.

Oral corticosteroids: Simultaneous administration of oral corticosteroids (e.g., prednisone) in the first two to four weeks of the course may quickly reduce inflammation and give the treatment a head start.1

Side Effects of Isotretinoin Accutane

Alternative Treatments for Acne Conglobata

Alternative treatments 

  • Tetracyclines: Tetracyclines are antibiotics sometimes prescribed as alternatives to isotretinoin, although their effectiveness in acne conglobata has not been established.1 Tetracyclines should never be combined with isotretinoin because of a risk of increased intracranial pressure.14
  • Cryotherapy: Cryotherapy includes applying a probe with a cooling agent onto cysts/nodules, and cooling down the lesions to temperatures at which they "die." The method may help to get rid of a few particularly stubborn nodules/cysts, but does not treat the causeof acne conglobata. In other words, cryotherapy does not comprehensively treat the disease, but only slightly masks it. 

    Clinical trial on cryotherapy in acne conglobata

    British Journal of Dermatology

    A study published in 1974 in the British Journal of Dermatology investigated the ability of cryotherapy to treat 25 patients with acne conglobata.13 Most lesions resolved within 7 - 10 days and did not return at the follow-ups 12 - 28 months later. 

  • Photodynamic therapy: Photodynamic therapy includes illuminating the affected areas of skin with red light after applying a special substance called a sensitizing agent. Like cryotherapy, photodynamic therapy does not treat all aspects of acne conglobata. In addition, in around one in six patients, photodynamic therapy worsens the symptoms of the disease.

    Clinical trial on photodynamic therapy in acne conglobata

    Photodermatology, Photoimmunology & Photomedicine

    A study published in Photodermatology, Photoimmunology & Photomedicine in 2013 reported treating acne conglobata on the face with red light photodynamic therapy.14 The investigators applied a sensitizing agent, called 5-aminolevulinic acid (ALA), onto facial skin and illuminated the cysts with red light for 20 minutes. After three procedures, 88% (28/32) of the patients had their cysts cleared. However, 16% (5/32) of the patients developed significant swelling and pain in the areas exposed to light, and the number of cysts increased.

Summary

Acne conglobata is a severe form of nodulocystic acne that usually affects young men and presents a combination of nodules, cysts, blackheads, abscesses, and draining sinuses. The disease is very stubborn and often lingers for months oryears. The studies show that isotretinoin works best for acne conglobata. However, only a doctor can determine the optimal treatment course for each individual case.

The Experts at Acne.org

Our team of medical doctors, biology & chemistry PhDs, and acne experts work hand-in-hand with Dan (Acne.org founder) to provide the most complete information on all things acne. If you find any errors in this article, kindly use this Feedback Form and let us know.

References:

  1. Schwartz, R. "Acne Conglobata". Emedicine.medscape.com. 2017. Web. 10 June 2017.
  2. Habif, T. Clinical Dermatology: A Color Guide to Diagnosis and Therapy (6th Edition). 231 - 233 (Saunders, 2015)
  3. Morris-Jones, R. & Meaden, A. ABC Of Dermatology. 92 (2014)
  4. Jansen, T., Lindner, A. & Plewig, G. Draining sinus in acne and rosacea. A clinical, histopathologic and experimental study. Hautarzt 46, 417 - 420 (1995)
  5. Jansen, T. & Plewig, G. Acne fulminans. Int J Dermatol 37, 254 - 257 (1998).
  6. Peck, G. et al. Prolonged Remissions of Cystic and Conglobate Acne with 13-cis-Retinoic Acid. New Engl J Med 300, 329 - 333 (1979)
  7. Meigel, W., Gollnick, H., Wokalek, H. & Plewig, G. Oral treatment of acne conglobata using 13-cis-retinoic acid. Results of the German multicentric study following 24 weeks of treatment. Der Hautarzt 34, 387 - 397 (1983)
  8. Schmidt, J. & Fanta, D. 13-cis-retinoic acid-an effective therapy for acne conglobata. Zeitschrift fur Hautkrankheiten 59, 279 - 287 (1984)
  9. Farrell, L., Strauss, J. & Stranieri, A. The treatment of severe cystic acne with 13-cis-retinoic acid. J Am Acad Dermatol 3, 602 - 611 (1980)
  10. Strauss, J. et al. Isotretinoin therapy for acne: Results of a multicenter dose-response study. J Am Acad Dermatol 10, 490 - 496 (1984)
  11. Leyden, J., McGinley, K. & Foglia, A. Qualitative and Quantitative Changes in Cutaneous Bacteria Associated with Systemic Isotretinoin Therapy for Acne Conglobata. J Invest Dermatol 86, 390 - 393 (1986)
  12. Hodgson, B. & Kizior, R. Saunders nursing drug handbook 2012. (W. B. Saunders Co., 2014)
  13. Leyden, J., Mills, O. & Kligman, A. Cryoprobe treatment of acne conglobata. Br J Dermatol 90, 335 - 341 (1974)
  14. Yang, G. et al. Short-term clinical effects of photodynamic therapy with topical 5-aminolevulinic acid for facial acne conglobata: an open, prospective, parallel-arm trial. Photodermatol Photoimmunol Photomed 29, 233 - 238 (2013).

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