Acne Fulminans - Causes, Risk, Diagnosis, and Treatment

What Are Acne Fulminans?

Acne fulminans, a severe skin disorder, is one of the worst forms of acne, accompanied by systemic symptoms. It is also known as acne maligna. It usually causes sudden onset ulceration and bleeding with pain. It also causes severe scarring. In addition to systemic symptoms like fever and polyarthritis (joint pain in more than five joints), it also causes bone lesions and abnormal bodily parameters. Acne fulminans do not respond to regular antibacterial therapy for acne but can be well managed through debridement and oral steroids.

What Causes Acne Fulminans?

• Though the exact cause of acne fulminans is not understood, the following conditions are being found to be the causative factors;

• In people with severe acne complaints, the use of high doses of Isotretinoin is linked to triggering acne fulminans.

• High levels of testosterone and the use of anabolic steroids can increase sebum secretion and also cause an increase in the population density of cutibacterium acnes (formerly propionibacterium acnes) in the skin. The antigens of these bacteria trigger an immunologic reaction in the body to cause acne fulminans.

• Genetic inheritance.

• Autoimmunity is also regarded as a cause of circulating immune complexes in some patients with acne fulminans.

What Syndromes Are Linked to Acne Fulminans?

• Acne fulminans can also be one of the parts of the following syndromes;

• Osteitis syndrome, hyperostosis, pustulosis, synovitis, and acne are collectively known as SAPHO.

• PAPA stands for pyogenic arthritis, pyoderma gangrenosum, and acne syndrome.

• Pyoderma gangrenosum, acne, and hidradenitis suppurativa syndrome (PASH).

• Pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa syndrome (PAPASH).

Is Acne Fulminans a Common Condition?

No. Acne fulminans is an uncommon condition, approximately only 150 cases have been discovered worldwide so far.

Who Is at Risk of Acne Fulminans?

It predominantly affects the adolescent male population of the white race aged between 13 and 22 years with a history of acne. These teen boys suddenly develop a large number of pimples on their back and around the chest region.

People with measles infection, late-onset congenital adrenal hyperplasia, and those taking anabolic steroids for bodybuilding are at increased risk, as the incidence of acne fulminans in such people has been previously reported.

What Are the Characteristic Features of Acne Fulminans?

• The acne is usually of sudden onset and large in number.

• The acne is painful, ulcerated, crusted, and hemorrhagic.

• Numerous inflammatory nodules are found on the trunk.

• Severe acne scarring.

• Inflamed lesions appear cystic.

• Fever.

• Malaise.

• Loss of appetite and weight.

• Pain in multiple joints (polyarthritis, especially of the hip, thighs, and knee joints).

• Erythema nodosum.

• Splenomegaly.

• Stooped posture.

• Failure to respond to usual antibiotic therapy for acne.

• Bone pain.

• Osteolytic changes in the bone.

• Though the characteristic feature of this condition is skin lesions, it is considered a systemic disease due to the involvement of several organs. Also, the affected people find it difficult and painful to walk due to arthralgia.

What Other Conditions Resemble Acne Fulminans?

Other conditions with similar characteristic features of acne fulminans include,

• Acne conglobata.

• Acne vulgaris.

• Acneiform eruptions.

• Pyoderma gangrenosum.

Which Is More Contagious: Boils or Acne?

• Acne itself is not contagious. It is a skin condition caused by excess oil production, bacteria, and inflammation. It is not spread from person to person through contact.

• Pimples, which are a symptom of acne, are also not contagious. They develop when cell debris and oil block the roots of hair, causing discomfort.

• Boils, on the other hand, can be contagious. They are usually caused by a bacterial infection of the underlying hair and scalp follicles.

• The bacteria-causing boils can spread through contact with the pus from the boil, especially if proper hygiene isn't maintained.

• So, between boils and acne, boils have the potential to be more contagious due to the bacteria involved and their spread through contact with infected materials.

What Investigations Help Diagnose Acne Fulminans?

After visually examining your lesions, your physician will order the following investigations to reach a diagnosis.

1. Complete blood count.

2. Erythrocyte sedimentation rate.

3. C-reactive protein.

4. Liver function test.

5. X-rays.

The following investigatory findings led to the diagnosis:

1. Increased white blood cell count (leukocytosis).

2. Anemia (low hemoglobin).

3. Elevated erythrocyte sedimentation rate.

4. Elevated C-reactive protein levels.

5. Osteolytic lesions of the bone.

How Can Acne Fulminans Be Treated?

The following treatment methods are found beneficial in treating acne fulminans:

Combination of Oral Steroids and Isotretinoin - Oral steroids like Prednisone, 0.5 to 1 mg/kg/day, will be prescribed for at least two weeks if only cutaneous lesions are present and for four weeks if systemic symptoms accompany them. The dose will be gradually tapered off. Meanwhile, isotretinoin 0.1 mg/kg/day will also be prescribed for four weeks. It is started with a low dose, eventually increased until desired effects are achieved, and then decreased towards the end of the treatment. The minimum dose of isotretinoin should not exceed 120 mg/kg (and 150 mg/kg in case of relapse).

Oral steroids with supplemental intralesional therapy are also preferred. A combination of Prednisone and Dapsone (50 to 100 mg/day) or Cyclosporine is also effective. Though antibiotics do not yield good results, broad-spectrum systemic antibiotics like 300 mg Clindamycin thrice daily and 750 mg Levofloxacin daily in addition to 1 mg/kg/day oral Prednisolone are found beneficial in some people. However, oral isotretinoin and steroids are always superior to an antibiotic-isotretinoin combination in treating acne fulminans.

Pulsed dye laser therapy is also beneficial but does not improve the pain. In acne fulminans patients with ulcerative colitis, Diaminodiphenyl Sulfone is beneficial. If unresponsive to the above treatment options, Infliximab (monoclonal antibody against tumor necrosis factor-alpha) is used.

Note - Women undergoing treatment with isotretinoin should avoid getting pregnant within a month of discontinuing isotretinoin.

What Is the Prognosis of Acne Fulminans?

The condition gets managed better with treatment. Though the lesions subside, the scars persist. The recurrence is very rare. Bone lesions also resolve, but the radiologic findings remain unchanged after successful treatment.

Conclusion:

As acne fulminans more profoundly affects the adolescent population, these adolescents eventually develop low self-esteem towards their appearance, as it greatly causes scarring, ulcerations, and crusts that seem unappealing. Suicidal ideation is also prevalent among the affected individuals due to unpleasant bodily lesions. This needs to be addressed, and prompt treatment should be sought.