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Acne Fulminans

Acne fulminans is a severe form of acne vulgaris that is even worse than acne conglobata. It also causes fever and may cause arthritis too. Find out more about this severe acne disease and how it is treated.
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Acne refers to a set of common skin diseases. However, the most common form of acne is acne vulgaris.

The severity of acne depends on a lot of factors but if left untreated, mild acne quickly progresses to moderate and then to severe acne.

During mild acne breakouts, there is very little skin inflammation and a few acne comedones such as whiteheads, blackheads and milia. When acne is not properly controlled at this stage, the comedones appear in greater numbers and are joined by inflammatory lesions such as papules and pimples.

When moderate acne progresses to severe acne, even more acne lesions appear and in greater numbers.

These new acne structures are caused by deeper damage to the dermis. They include nodules and cysts. Nodules and cysts are similar. They are hard, lumps below the skin surface. Cysts differ from nodules because they are still filled with pus and when they rupture, they can cause even wider and deep damage to the skin.

Severe acne can leave permanent scars on the skin. At this stage, acne is difficult to treat and require even more aggressive treatment options.

What is Acne Fulminans?

Acne fulminans is one of the severe forms of acne vulgaris.

Normal severe acne vulgaris is called cystic acne. When cystic acne spreads and worsens, it turns into acne conglobata. Acne fulminans is an even more severe form of acne. It results from unsuccessfully treated acne conglobata.

Acne fulminans was previously called acute febrile ulcerative acne. It is also called acne maligna.

Acne fulminans is a rare form of acne caused by immunological response to high androgen levels and antigens released from the acne-causing bacterium, Propionibacterium acnes.

In some people, androgens are the primary trigger. This is especially true for those receiving testosterone therapy or those using high amounts of anabolic steroids. With increased androgen levels in the body, the sebaceous glands are primed to produce more sebum and the excess sebum serves as ideal growth environment for Propionibacterium acnes.

The antigens released by this bacterium then triggers antibody reaction from the immune system. This fact has led some experts to describe acne fulminans as an autoimmune disease.

The immunological reaction experienced during acne fulminans breakout is a type III or IV hypersensitivity reaction.

Acne fulminans may also have a genetic cause. The exact genetic markers for the acne are still unknown but of the few documented cases of acne fulminans, there have been sets of identical twins that presented with identical symptoms and identical progression of acne fulminans.

Besides testosterone, isotretinoin has also been known to trigger acne fulminans by increasing immunological response to the antigens of P. acnes.

Appearance and Progression of Acne Fulminans

Acne fulminans mostly affects young males between the ages of 13 and 22 with a history of acne. Acne fulminans has a sudden onset.

Defining Features of Acne Fulminans
  • Severe inflammatory and ulcerating acne nodules and cysts on the chest and back
  • Severe and extensive acne scars
  • Fluctuating fever and malaise
  • Polyarthritis

There are some distinguishing features between acne fulminans and acne conglobata. For example, acne fulminans does not respond to antibiotics therapy while acne conglobata does.

Furthermore, a quick physical examination can differentiate the two forms of severe acne.

Acne fulminans produces large, painful nodules which form into ulcers joined by oozing scabs. There are no non-inflammatory cysts and comedones on the skin affected by acne fulminans as there are for acne conglobata.

Acne fulminans may cause bone inflammation and joint pains that make walking very difficult. The arthritis caused by acne fulminans mostly affects the joints of the clavicles, ribs, knees, thighs and hips.

To diagnose acne fulminans, there are specific sets of blood markers to look for.

Since acne fulminans involves an immunological response, the white blood cells count is always raised and the amount of immune complexes circulating in the blood will become high. Therefore, confirmatory laboratory tests look for increased leukocyte number and activity.

Anemia, increased sedimentation of red blood cells and high concentrations of proteins in the urine are also signs of acne fulminans.

Bone imaging can also contribute to the diagnosing of acne fulminans because it affects the bone. In half the patients affected by this form of severe acne, bone lesions can be seen on bone scans. In 25% of patients, bone inflammation is observed.

Acne Fulminans and Arthritis

A number of studies have been conducted to examine the effect of acne fulminans on bones. All of these studies agree that this severe form of acne damages bones and joints.

In one study, published in 1996 in Acta Dermato-Veneroligica, 3 out of every 4 patients experiencing acne fulminans experienced increased degradation of type I collagen.

Type I collagen is the most abundant collagen found in the skeleton.

In a review of scientific literature on the subject published in the Journal of the American Academy of Dermatology in 1989, acne fulminans was associated with decreased estradiol levels. Because estradiol is an anti-acne hormone that also protects against the demineralization of bones, low estradiol levels means that androgens can go unchecked and worsen acne while the bone is stripped down.

Acne fulminans can also be a component of an even bigger musculoskeletal disorder called SAPHO.

SAPHO syndrome is a disease complex that describes a number of inflammatory bone disorders which can also be accompanied by skin problems.

SAPHO stands for Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis.

What SAPHO means

  • Synovitis – inflammation of the synovial membrane of the joints
  • Acne – common skin disease causing inflammatory and non-inflammatory lesions
  • Pustulosis – highly inflammatory skin disorder causing large, pus-filled blisters on the palms of hands and soles of feet
  • Hyperostosis – excessive bone growth
  • Osteitis – inflammation of the bones

There are two types of severe acne associated with SAPHO. These are acne fulminans and acne conglobata.

While acne conglobata mostly affects the bones of the limbs and spines, acne fulminans mostly affects the bones of the clavicle and sternum. However, acne fulminans can still cause arthritic pain in the limbs.

The cause of SAPHO is unknown but it is believed to have a genetic component.

How Acne Fulminans Is Treated

Acne fulminans may first be treated with high dose, oral antibiotics and NSAIDs (non-steroidal anti-inflammatory drugs). However, most acne fulminans sufferers do not respond to broad-spectrum antibiotics and NSAIDs may be too mild to control the extensive inflammation.

In fact, treating with antibiotics is one of the reasons acne fulminans becomes even more severe.

Instead of wasting time with a treatment course with poor results, experts recommend a combination of isotretinoin and oral steroids as first-line drugs for acne fulminans.

In this treatment, the patient is started on low dose isotretinoin and high dose steroids. The doses of the steroids are then reduced while isotretinoin doses are increased over the period of treatment.

Care should be taken with these two drugs since they have serious side effects. Even more care should be paid to isotretinoin since it has been shown to increase the risk of suicidal tendencies and such tendencies are common in people affected by acne so severe as acne fulminans.

Patients who do not respond well to this combination can be treated with infliximab, a monoclonal antibody that inhibits TNF-alpha (tissue necrosis factor-alpha).

Pulsed dye laser treatment has also been used successfully to treat acne fulminans.

Once the acne fulminans has been completely cleared off the skin, relapse rarely occurs. However, for the very few patients who show signs of relapse, slightly higher doses of isotretinoin can be used for a second course of therapy.

Sources


http://dermnetnz.org/acne/acne-fulminans.html

http://www.ncbi.nlm.nih.gov/pubmed/2521643

http://www.ncbi.nlm.nih.gov/pubmed/8740265

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