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Acne conglobata is a severe form of acne vulgaris. It can cause extensive and permanent scarring on the skin. Find out how it occurs, what causes it, who is most at risk and how it is treated.
Acne vulgaris is a common skin disease which shows as blemishes on the skin. Depending on the severity of the breakout, acne can be classified as mild, moderate or severe.
When the acne breakout is mild or moderate, there is only a little skin inflammation and a lot of papules, pustules and acne comedones such as whiteheads and blackheads. However, severe acne is characterized by the appearance of a lot of inflammatory acne bodies.
The two major presentations of severe acne are nodules and cysts.
Nodules and cysts are caused by deep infection and damage to the dermis. Unlike comedones, papules and pustules which are superficial acne lesions, nodules and cysts can cause permanent damage to the skin.
Nodules are seen as large, hard lumps underneath the skin surface. Cysts look like nodules but they are filled with pus which can still rupture to cause even deeper infections.
Nodulocystic or cystic acne is only one form of severe acne vulgaris.
Acne conglobata is a form of severe acne vulgaris. It is described as an inflammatory skin disease presenting with acne comedones, nodules, inflamed pus-filled tissues and draining sinus tracts.
Acne conglobata affects mostly men around between the ages of 18 and 30.
It can be a progression of an active but uncontrolled acne or the resurgence of a previously controlled acne.
The cause of acne conglobata is unknown but since it appears mainly in men, it is believed to be caused high levels of androgens such as testosterone. Another support for this theory comes from the fact that acne conglobata is commonly seen in men who abuse anabolic steroids and those who have recently stopped testosterone therapies.
People with androgen-releasing tumors, women with PCOS (polycystic ovarian syndrome) or those with Cushing’s syndrome have a very high risk of developing acne conglobata. Those also classified to be at risk of developing this form of severe acne include those who are in remission from autoimmune diseases and those exposed to halogens or hydrocarbons.
Studies have also shown that acne conglobata runs in some families.
This points to a genetic cause and in fact, defects in XYY karyotype or chromosomal composition have been associated with severe forms of acne conglobata.
There is a strong link between the disease and specific human leukocyte antigen (HLA) phenotypes.
Yet another cause of acne conglobata is believed to be changed sensitivity of the skin to the acne-causing bacteria such as Propionibacterium acnes. It could be that some people suddenly developed a heightened sensitivity to this bacterium which then quickly spreads to other parts of the body.
This last theory is the basis of antibiotic use in the treatment of acne conglobata.
Acne conglobata starts with blackheads appearing on the face in clusters of 2 – 3 comedones. These clusters then spread to the neck, chest, back, upper arms, thighs and the buttocks.
Blackheads are acne comedones which leave dark spots on the skin. They are formed from partially blocked pores which are filled with sebum, dead skin cells and bacteria. The dark spots left on the skin are due to the leakage of melanin, the skin pigment. When melanin is exposed to the air, it oxidizes and turns black.
Following the spread of blackheads, pimples also congregate around the blackheads.
These pimples quickly fill with pus and grow in size. Once engorged, they become raw and sensitive to touch. While they can be easily popped, they are quite painful and further aggravation is not advised.
However, the large, engorged pimples of acne conglobata rupture and spread further. Each ruptured pimple is then filled again with pus and become even larger. These larger pimples soon form into nodules and cysts.
By interconnecting with each other, these fused nodules cover large areas of the skin.
Even as the body tries to heal the earliest nodules, the large lump extends at the edges. This leads to the formation of scabs at the center of advancing inflammatory nodulocystic structures.
Eventually, these wounds do heal but leave extensive and permanent scars on the skin. Acne conglobata can leave either pitted, atrophic scars (with tissue loss like the typical ice pick acne scars) or raised, hypertrophic (with keloid structures formed from excess collagen as the body overcompensates for tissue loss).
While acne conglobata is a rare disease, acne arthritis due to acne conglobata is even rarer. It has only been reported in single cases.
There are two types of acne that are known to be associated with musculoskeletal diseases. These are acne conglobata and acne fulminans. Acne vulgaris, the common type of acne does not affect the muscles, bones or joints.
Acne arthritis mostly affects young males who also make up the subset of the population affected by acne conglobata. In almost all patients with acne arthritis, there is a common genetic marker that can be used to confirm the disease.
This genetic marker is human leukocyte antigen B27 negative or HLA-B27 negative.
Cases of acne arthritis show that acne conglobata is mostly associated with peripheral and axial arthritis (arthritis of the bones of the limbs and spine) while acne fulminans mostly causes multiple, painful bone lesions especially in the clavicles and sternum.
SAPHO is the medical name for acne arthritis. It is a name taken from the 5 symptoms that define this condition.
SAPHO syndrome describes a number of inflammatory bone disorders that may or may not be associated with skin diseases.
The cause of SAPHO syndrome is unknown except that most people who suffer from it inherit the blood marker, HLA-B27.
Isotretinoin is the most common drug prescribed for acne conglobata. It can also be combined with oral steroids such as prednisone.
Since isotretinoin cannot be used in certain situations and also because of its extensive side effects, tetracycline antibiotics are sometimes prescribed as alternatives. These include tetracycline, minocycline and doxycycline. Erythromycin can also be prescribed.
For cases resistant to tetracycline antibiotics, dapsone is used instead. There have been cases of successful treatment of acne conglobata with a combination of dapsone and isotretinoin. However, given that both drugs do have serious side effects, treatments with such combination must be closely monitored.
When oral drugs fail to stop acne conglobata, topical treatments are advised.
The usual treatment for unresponsive cases is carbon dioxide laser treatment combined with topical tretinoin therapy. External beam radiation is another treatment course useful for treating this form of acne.
When acne conglobata is present as a component of SAPHO syndrome, drugs such as sulfasalazine and methotrexate are used. Newer drugs such as infliximab and etanercept are also used and may be combined with isotretinoin.
Where all drug interventions fail, surgery is the last recourse.
Surgical care for acne conglobata involves the removal of large, hemorrhagic nodules by suction. Interconnected, large nodules may also be surgically removed to stop their spread. Injecting triamcinolone directly into the lesion may also be helpful in stopping the progression of the skin disease.
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