What is acne conglobata

Acne conglobata also called severe nodulocystic acne, is a rare and unpleasant severe form of nodulocystic acne in which there are interconnecting abscesses and sinuses (channels under the skin) ¹. These result in unsightly hypertrophic (thick) and atrophic (thin) scars. There are groups of large macrocomedones and cysts that are filled with smelly pus 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 ².

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 acne conglobata induces polyporous comedones and non-inflammatory cysts, whereas acne fulminans does not ³.

Overall, acne conglobata 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 ⁴.

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.

Acne conglobata is occasionally associated with:

• Other disorders associated with follicular occlusion, particularly hidradenitis suppurativa, a condition in which similar boil-like lesions and scars occur in the armpits, groins and under the breasts.
• Nodulocystic acne may very rarely be a manifestation of a genetic disorder, PAPA syndrome.

Acne conglobata is best managed by a dermatologist as it requires more aggressive therapy with close follow up.

Treatment of nodulocystic acne can be challenging and may require a combination of medicines. Topical treatment is usually ineffective.

The recommended treatment for nodulocystic acne is isotretinoin, which should be commenced early to prevent scarring. The treatment is required for at least five months, and further courses are sometimes necessary.

Patients with acne conglobata often need additional treatments, such as:

• Oral antibiotics
• Intralesional steroids
• Systemic steroids
• Hormonal therapy in women

Acne conglobata causes

It is believed that Propionibacterium acnes, the same organism implicated in acne vulgaris (common acne), may play an important role in acne conglobata by changing its reactivity as an antigen ³. The hypersensitivity to Propionibacterium acnes 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 ⁵.

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.

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.

Acne conglobata 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 ⁶. Lesions begin to form in the late teen years, and the disease often persists into the late twenties and, sometimes, early thirties.

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) ⁷. Whiteheads, papules, and pustules may be present as well, but they are not a dominant feature.

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.

• 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 diagnosis

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.

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.

Acne conglobata treatment

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.

• 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.

References

1. Nodulocystic acne. https://www.dermnetnz.org/topics/nodulocystic-acne
2. 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.
3. Hafsi W, Badri T. Acne Conglobata. [Updated 2019 May 2]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK459219
4. Scheinfeld N. Diseases associated with hidranitis suppurativa: part 2 of a series on hidradenitis. Dermatol. Online J. 2013 Jun 15;19(6):18558
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 Jul;173(7):1903-1906.
6. Habif, T. Clinical Dermatology: A Color Guide to Diagnosis and Therapy (6th Edition), Saunders, 2015. 231 – 233
7. Schwartz, R. “Acne Conglobata”. Emedicine.medscape.com. 2017. Web. 10 June 2017
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.