What are the other Names for this Condition? (Also known as/Synonyms)

• AC (Acne Conglobata)
• Nodulocystic Conglobata Acne

What is Acne Conglobata? (Definition/Background Information)

• Acne is a common skin condition that causes inflamed red spots/lesions to appear on the face, neck, shoulders, and other regions. The condition occurs when the tiny follicles (openings) of the sebaceous glands become clogged with dead cells and oil
• Acne Conglobata (AC) is a severe form of acne that results in the formation of nodules in the body, mostly in the face and neck region. It is described as a type of nodulocystic acne that forms in young adults and teenagers
• The cause of the condition is unknown, but it may be influenced by factors such  a positive family history of Acne Conglobata. In addition, elevated androgen levels, either endogenous or from deliberate use of androgens, can be contributory. Constant exposure to high heat and humidity can exacerbate the condition, in which case the term tropical acne can be used
• Over time, the nodules of Acne Conglobata can get infected, rupture, bleed, and ooze pus. The skin lesions can lead to severe scarring and disfigurement. Even in the absence of infection, the immune/inflammatory system responds to the process as it would to a true infection
• Acne Conglobata may be treated with antibiotics, laser therapy, and immunotherapy. Isotretinoin is another mainstay of treatment. The prognosis of the condition depends upon several factors, including on the severity of the signs and symptoms

Who gets Acne Conglobata? (Age and Sex Distribution)

• Acne Conglobata is an uncommon condition that is mainly seen to develop in teens and young adults between the age of 15-30 years
• The skin condition can rarely affect infants and children
• Both males and females are at risk, though Acne Conglobata is more common in males
• There is no racial or ethnic predilection, and the condition may be seen worldwide

What are the Risk Factors for Acne Conglobata? (Predisposing Factors)

The following are the risk factors for Acne Conglobata:

• Use of performance-enhancing anabolic steroids
• Testosterone-secreting tumors
• The condition can run in families, and hence, those with a positive medical history may develop Acne Conglobata

Rare[JK1] cases of Acne Conglobata (the “A” in the acronyms) have been associated with the following genetic disorders:

• Pyogenic arthritis, Acne Conglobata and pyoderma gangrenosum (PAPA Syndrome)
• Pyoderma gangrenosum, Acne,  Conglobata,and Suppurative Hidradenitis (PASH Syndrome)
• Pyoderma gangrenosum, Acne Conglobta, and Ulcerative Colitis (PAC Syndrome)
• Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis (PsAPASH Syndrome)
• Pyogenic Arthritis, Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis (PAPASH Syndrome)
• Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis ,and Ankylosing Spondylitis (PASS Syndrome)
• The SAPHO syndrome (Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis syndrome) has Acne Conglobata as a feature

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Acne Conglobata? (Etiology)

• The cause of Acne Conglobata is unknown in a majority of cases. It occurs due to a sudden worsening of existing acne due to various factors
• Acne Conglobata is thought to be a longstanding (chronic) type of acne vulgaris, which is the simplest form of acne
• In most of the Acne Conglobata-associated syndromes listed above, mutations in the PSTP1 gene have been linked
• Some researchers have proposed that it may be due to an immune reaction of the body to the bacterial organism Propionibacterium acnes. Propionibacterium acnes is a normal resident of the sebaceous glands

What are the Signs and Symptoms of Acne Conglobata?

The signs and symptoms of Acne Conglobata are as follows:

• Exacerbation of papular acne and pustular acne (or infected acne), which may take place due to a variety of factors
• The face, neck, and torso are the usual sites of Acne Conglobata skin lesions
• These skin lesions are highly inflammatory
• Nodules form on the body that get infected and rupture
• Ruptured lesions can bleed; the formation of pockets of pus and abscesses may occur
• Formation of skin ulcers with the discharge of pus
• Scarring of skin: Nodules and skin cysts that are deep into the skin may cause scarring of the skin, which may be permanent

How is Acne Conglobata Diagnosed?

Acne Conglobata is diagnosed through a physical examination by the physician or dermatologist.

• The healthcare provider may also ask many questions related to the individual’s age, family medical history, current medications, cosmetics, body lotions used, other medical conditions, infections, etc.
• Test for blood levels of testosterone and other androgens
• Tests to rule-out polycystic ovarian syndrome (PCOS)
• 24-hour urinary cortisol levels
• If secondary infections develop, then a culture test (culture of skin biopsy) may be performed
• Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed), and microscope findings, the pathologist arrives at a definitive diagnosis

A differential diagnosis may be undertaken to rule-out the following skin conditions:

• Acne fulminans
• Acne rosacea
• Acne vulgaris
• Acneiform eruptions
• Folliculitis
• Sporotrichosis (a form of fungal infection)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Acne Conglobata?

Some of the complications of Acne Conglobata include:

• Severe psychological stress
• Cosmetic disfigurement requiring extensive surgical treatment (restoration)
• Secondary bacterial infections
• Development of acne fulminans, which may be described as a very severe form of Acne Conglobata
• Complications of the underlying condition or disorder, if any

How is Acne Conglobata Treated?

Treatment measures for Acne Conglobata may include the following:

• Oral and topical antibiotics
• Topical retinoids
• Topical benzoyl peroxide
• Isotretinoin
• Immunomodulatory treatments to decrease the inflammatory process
• Treatment of any associated or identified contributing factors

After the resolution of the active disease process, treatments to improve the scarring can be done. These include chemical peels, laser abrasion, dermabrasion, punch excision, and regular excision of scars.

How can Acne Conglobata be Prevented?

Currently, Acne Conglobata is a rare condition that has no known preventive measures. Nevertheless, controlling some of the risk factors may help avoid the skin condition. These include:

• Avoiding the use of anabolic steroids unless for a medical condition
• Undertaking adequate treatment of any tumors that secrete the hormone testosterone

What is the Prognosis of Acne Conglobata? (Outcomes/Resolutions)

• Acne Conglobata is a severe form of nodulocystic acne. The prognosis of the condition depends upon the severity of the signs and symptoms, response to treatment, and the severity of the underlying condition
• Severe cases of Acne Conglobata may lead to severe facial or skin disfigurement resulting in emotional trauma, stress, and reduced self-esteem

Additional and Relevant Useful Information for Acne Conglobata:

• There is no evidence to prove that oily foods and chocolate-based products influence Acne Conglobata
• Cleaning the skin too hard with strong chemicals or soaps may aggravate the skin condition
• The presence of dirt on the body is not a causative factor for Acne Conglobata; but it helps to be clean and hygienic