What is Acne Conglobata?
Acne conglobata (AC) is a rare but severe form of acne. It presents with painful, disfiguring lumps and cysts that often connect with each other. These can appear on the face, shoulders, back, chest, upper arms, buttocks, and thighs. The spots often appear in groups, and the cysts can have a bad-smelling discharge that can leak onto the skin. It will eventually lead to scarring and physical changes to the face or other affected parts of the body.
AC can appear after a sudden worsening of pustular acne, or it can gradually occur after acne that had been inactive for a long time reappears. It belongs to a group of related diseases called the follicular occlusion tetrad. This group also includes diseases such as dissecting cellulitis, pilonidal disease, and hidradenitis suppurativa (HS). These conditions are all challenging to treat and often require intensive treatment. AC in particular, due to the degree of inflammation and deep cysts, is much more difficult to treat than normal acne.
AC can occur alone or with other conditions, such as SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis), PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum, acne), PASH syndrome (pyoderma gangrenosum, acne, suppurative hidradenitis), or PAPASH syndrome (pyogenic arthritis, pyoderma gangrenosum, acne, suppurative hidradenitis). Acne fulminans can sometimes occur, which is a severe form of acne that comes on suddenly with oozing and open sores. This is related to these syndromes or the use of a drug called isotretinoin. Acne fulminans may also come with other symptoms such as fever, feeling unwell, and weight loss. It can also appear suddenly after starting isotretinoin or in relation to the use of anabolic steroids.
What Causes Acne Conglobata?
Acne is a skin condition that develops as a result of various factors working together. These include overproduction of a type of skin cell, excessive oil production influenced by hormones, and inflammation. A type of skin-dwelling bacteria called Cutibacterium acnes, which normally live deep in our oil glands, also contribute to the process that leads to acne.
An initial stage in acne development is a tiny blockage in our skin’s hair follicles, also known as a microcomedo. This happens when skin cells accumulate in the follicle instead of shedding normally. This accumulation forms a plug that traps more skin cells and oil behind it. As this build-up continues, it turns into an acne spot, or comedo, which can further develop into a swollen bump known as a papule. If the follicular wall breaks, it causes intense inflammation, leading to the formation of larger acne lesions, such as nodules or cysts. Acne conglobata, a severe type of acne, consists of numerous connected comedones, cysts, nodules, and draining sinus tracts, which can get infected often.
The activity of our skin’s oil glands is primarily regulated by hormones such as testosterone. The increase in these hormones at the onset of puberty leads to increased oil production. Interesting to note, people with acne generally produce more oil and have a different composition of the oil. For example, they have higher levels of a compound named squalene, which can lead to inflammation, and lower levels of linoleic acid, whose reduction makes the skin more prone to forming comedones and getting inflamed.
While it was previously thought that an overgrowth of Cutibacterium acnes led to acne, recent studies suggest that it isn’t the amount, but rather the specific subtype of the bacteria that’s responsible for inflammation in acne. Certain subtypes of the bacteria may actually protect the skin, but if there’s an imbalance with other subtypes, it could lead to acne. This imbalance is especially seen in severe acne cases like acne conglobata, although the exact reasons are still not clear.
Certain triggers can provoke severe acne like acne conglobata, including taking thyroid medication, exposure to certain compounds called halogenated aromatic hydrocarbons, androgens (like those produced by certain tumors), anabolic steroids, and hormonal agents. In some cases, acne conglobata is seen in individuals who stop using anabolic hormones. Interestingly, some people with acne conglobata have an unusual genetic condition called the XYY karyotype.
Risk Factors and Frequency for Acne Conglobata
Acne conglobata is an uncommon type of acne. It tends to affect men more than women. This pattern is largely thought to be due to the use of anabolic steroids by athletes, based on reports from the last 30 years. The exact prevalence of acne conglobata outside of the United States isn’t known. Typically, young adults are the ones who get acne conglobata, whereas children and older adults rarely have this condition. The condition usually starts in people’s twenties or thirties. It’s unclear whether this type of acne is more common in any particular racial group.
Signs and Symptoms of Acne Conglobata
Acne conglobata is an uncommon and severe type of acne. It’s marked by painful, large, interconnected comedones (pimples), cysts, and nodules, accompanied by deep abscesses. This acne frequently appears on the face, shoulders, back, chest, upper arms, buttocks, and thighs. The pimples often group in threes, and the cysts usually contain smelly pus that is released onto the skin. This type of acne is a chronic inflammatory disease that results in scars and disfigurement.
People with acne conglobata might have a sudden worsening of pustular acne, or a gradual return of acne that has been inactive for many years. A physical examination will typically show severe acne with painful, dome-shaped nodular lesions. Once these nodules break down, they release a foul-smelling pus. After this pus drains, the lesion crusts over and forms large irregular scars.
- Severe, painful acne
- Dome-shaped nodular lesions
- Foul-smelling pus
- Crusting of lesions followed by scar formation
A defining characteristic of this condition is the presence of dual or groupings of blackheads found on the trunk, neck, upper arms, and buttocks.
Acne conglobata can develop following the use of androgenic anabolic steroids, especially common in bodybuilders. Affected individuals may present with severe acne and facial scarring, and it’s critical to determine whether these individuals have a history of anabolic steroid use, as stopping these agents is essential for proper treatment.
Moreover, people with conditions like hidradenitis suppurativa and pyoderma gangrenosum may also develop acne conglobata. In individuals where the condition emerges post-puberty, the acne nodules tend to merge and worsen over time. The active formation of nodules usually continues for the first three decades of life, after which they usually quiet down.
Testing for Acne Conglobata
The decision whether an infection is present is usually made based on physical symptoms, and a sample of the discharge should be sent for lab testing. If the discharge is foul-smelling, it’s important to start taking appropriate antibiotics right away (such as minocycline or doxycycline), as advised below. There’s no need to wait until the lab results come back before starting antibiotic treatment.
Treatment Options for Acne Conglobata
The best way to treat severe acne, also known as acne conglobata, is to use retinoids like isotretinoin for a period of 20 to 28 weeks, sometimes even longer. For the first 2 to 4 weeks, doctors may add a steroid called prednisone to the treatment. Steroids have been shown to be helpful when the acne gets worse at the beginning of the isotretinoin treatment or if there are symptoms affecting the whole body.
Sometimes, isotretinoin can trigger a severe form of acne with oozing and painful skin ulcers, called acne fulminans, which can come with fever, tiredness, loss of appetite, and weight loss. In such cases, the isotretinoin treatment should be temporarily stopped and switched to prednisone for a period of 2 to 4 weeks. After that, your doctor can restart isotretinoin at a lower dose (0.1 mg/kg per day), while continuing the prednisone treatment for an additional four weeks. Finally, your doctor will slowly increase the isotretinoin dose while gradually reducing the prednisone dose.
While topical (on-skin) retinoid treatments are less effective than oral (by-mouth) retinoids, they can be used in combination with oral treatments. However, retinoids are known to potentially harm unborn babies, so for women who could potentially become pregnant, good contraception methods are necessary.
There are other treatment options as well. The antibiotics minocycline or doxycycline can be used, taking 100 mg doses twice a day. Dapsone, taken between 50 and 150 mg per day, can also be an option for those who don’t respond to other treatments, although doctors need to carefully monitor the blood for specific issues related to this drug. In some cases, doctors may recommend the use of treatments that modify the immune response, such as infliximab, adalimumab, and etanercept. Laser therapy can also be effective, though this has only been confirmed in a few reports. In really severe cases, when nothing else seems to work, doctors might consider the use of external beam radiation.
In terms of surgery, when there are large lumps that are tender to touch (nodules), they can be drained. There are other options as well, including the use of cryotherapy (cold treatment), injecting a medicine into the acne (intralesional triamcinolone), or even surgical removal of the large nodules. Once the acne is under control and the skin has healed, dermal fillers can be used to improve the scars. Several types of fillers are available, some of which have been specifically approved for treating acne scarring.
What else can Acne Conglobata be?
When doctors are trying to diagnose acne conglobata, there are several similar conditions they might consider as alternatives:
- SAPHO syndrome, which stands for synovitis, acne, pustulosis, hyperostosis, osteitis
- PAPA syndrome, or pyogenic arthritis, pyoderma gangrenosum, and acne
- PASH syndrome, which stands for pyoderma gangrenosum, acne, and suppurative hidradenitis
- PAPASH syndrome, which includes pyogenic arthritis, pyoderma gangrenosum, acne, and suppurative hidradenitis
- Acne fulminans, a severe form of acne
- Acne vulgaris, the medical term for common acne
- Acneiform papulonodules, which are small, raised skin bumps that resemble acne
- Bromoderma, a skin condition caused by excess bromine
- Iododerma, a skin reaction to too much iodine
- Rosacea fulminans, a sudden and severe onset of rosacea, another skin condition
It is really crucial for doctors to consider all these possibilities so that they can make the right diagnosis.
What to expect with Acne Conglobata
Acne conglobata can result in significant physical changes, which often lead to the person feeling disfigured. This severe scarring can seriously impact mental health, causing individuals with this condition to feel socially isolated. Furthermore, acne conglobata has commonly been linked with heightened levels of anxiety and depression in many patients.
Possible Complications When Diagnosed with Acne Conglobata
Most people who suffer from a severe type of acne called acne conglobata often feel embarrassed about their skin condition. This might lead to them feeling unsocial and isolated. It’s not unusual for them to have thoughts about suicide. Therefore, it’s very important for these individuals to receive mental health support. They should be guided to consult their regular doctor or a mental health professional to help manage any potential feelings of depression.
Preventing Acne Conglobata
Once a person is diagnosed with a severe type of acne called acne conglobata, it’s essential they receive mental health support. This is due to the emotional distress that comes with the changes in their physical appearance, which can lead to feelings of depression or anxiety. To cope, these individuals might try to cover their skin blemishes with clothing. Unfortunately, this can result in a warmer and more humid environment that can actually make the skin condition worse. Therefore, it’s important for patients to also learn about proper skin care routines and undergo counseling to help manage their condition better.
