Acne fulminans (acne maligna, acute febrile ulcerative acne) is a rare skin condition in which a patient has acute, painful, ulcerated and bleeding skin lesions. This form of acne may also be accompanied by systemic symptoms such as fever, joint pain and bone pain. The condition is usually resistant to the usual antibiotics that a doctor prescribes to treat acne. Other medications and laser therapy reduce the severity of the lesions. In addition, treatment of psychological problems resulting from the condition is often necessary. Despite treatment, many patients with this skin disease experience scarring.

• Epidemiology
• Causes of acne fulminans
• Risk factors of skin disease
• Skin disease as part of a syndrome
• Symptoms

• Skin
• Systemic signs

• Diagnosis and examinations
• Treatment of skin condition

• Medicines
• Laser treatment
• Psychological support

• Prognosis of severe lumps on skin and joint pain

Epidemiology

The skin disease acne fulminans occurs very rarely. Only a few hundred patients have been described in the medical literature (the exact year of the description of the disease has not been found in the literature consulted as of October 2020). Most often young men are affected. They often have a white skin color and are between 13 and 22 years old. Most men have a history of acne (skin disease with inflammation of sebaceous glands). There have also been isolated reports of acne fulminans in women. The condition has been reported worldwide. Due to earlier and better treatment of acne, the skin disease is becoming less common in October 2020.

Causes of acne fulminans

Acne fulminans is a rare skin condition with an unknown cause in October 2020. Some theories are known. MedicationIsotretinoin, a medication that the doctor prescribes to treat severe acne, ironically may lead to acne fulminans (the skin disease is then a form of acne medicamentosa). Anabolic steroids increase sebum levels and the density of the bacteria Propionibacterium acnes (P. acnes) . Bodybuilders who use anabolic steroids may develop acne fulminans in a very short time. The density of P. acnes or related antigens activates the immune system and then leads to acne fulminans.TestosteroneElevated testosterone levels may also play a role in the development of the skin disease. That is why men are more likely to suffer from the skin condition.Autoimmune diseaseOther physicians suspect that acne fulminans is an autoimmune condition because circulating immune complexes are present in some patients with acne fulminans.GeneticsAcne fulminans also has a genetic component, but the inheritance pattern is unknown as of October 2020.

Risk factors of skin disease

Stress, infections and increasing heat of the environment are possible risk factors of the disease. More scientific research is needed in October 2020 to scientifically support these triggering factors.

Skin disease as part of a syndrome

The skin condition may be part of a syndrome such as:

• the PAPASH syndrome. This stands for pyogenic arthritis (PA), pyoderma gangrenosum (P), acne (A) and hidradenitis suppurativa syndrome (SH).
• the PAPA syndrome. This stands for pyogenic arthritis (PA), pyoderma gangrenosum (P), acne (A)
• the PASH syndrome. This stands for pyoderma gangrenosum (P), acne (A) and hidradenitis suppurativa syndrome (SH)
• the SAPHO syndrome. This stands for synovitis (S), acne (A), pustulosis (P), hyperostosis (H) and osteitis syndrome (O)

Symptoms

Skin

Acne fulminans usually has a very sudden onset that worsens severe acne. Acne fulminans is similar to the symptoms of acne conglobata (lumps and pustules on the face, trunk and back). A patient has numerous inflammatory lumps, (bleeding) skin ulcers, scabs, bumps, pustules and/or abscesses on the trunk, face, neck, and/or upper back. The red and purple-red skin lesions are often painful to the touch. A pus discharge, possibly with blood and skin ulcers are among the possible signs. Unlike acne conglobata, blackheads are not present.

Systemic signs

Patients may also complain of swollen lymph nodes (particularly in the neck), a generally ill feeling, fever, weight loss, loss of appetite, bone pain and pain in multiple joints (usually the knees, hips and pelvis). Patients with acne fulminans who have systemic symptoms have a stooped posture due to bone and joint pain. Painful enlargement of the spleen (splenomegaly) and erythema nodosum (skin infection with painful nodules) are sometimes associated with the disease.

Diagnosis and examinations

Most patients have a history of (severe) acne. They tried standard antibiotics for this, but this treatment was ineffective. Because the condition may be associated with systemic symptoms, the doctor should perform a complete blood count. Because many patients also experience bone and weight pain, the doctor often uses imaging tests such as an X-ray and a bone scan. Furthermore, the doctor looks at the history of severe acne, the worsening of the inflammation of the symptoms and the presence of systemic complaints. Based on these clinical signs and the other research results, he is able to confirm the diagnosis of acne fulminans.

Treatment of skin condition

Medicines treat the skin disease / Source: Stevepb, Pixabay

Medicines

The patient should avoid agents that may worsen the disease such as anabolic steroids. Acne fulminans also does not respond to the traditional antibiotics that a doctor uses to treat classic acne. The recommended treatment for acne fulminans is a combination of corticosteroids (powerful anti-inflammatories that suppress the immune system) and isotretinoin. Women of childbearing age should be very careful when using retinoids, because they may cause birth defects. After stopping the drug, a woman should not become pregnant for at least one month. The use of extra-strength topical (applied to the skin) corticosteroids may result in a faster response. Some doctors also experiment with biological medicines. Scars are common. Other (combinations of) medications can also be used. Finally, the patient may also take paracetamol or other painkillers to relieve pain and reduce fever. Once healing occurs, the lesions reduce in size, are less tender and painful, and have minimal inflammatory response.

Laser treatment

Pulsed dye laser is moderately effective for treating acne fulminans, but adverse effects and pain remain a problem. Because the lesions are often large, multiple treatments are required and scarring and hypopigmentation (lighter skin color) are common complications.

Psychological support

Acne fulminans is a serious systemic condition with disfiguring skin lesions. The majority of patients experience psychological problems and a reduced quality of life due to the appearance of the skin. They cannot participate in sports or social activities. Social isolation is a common problem in patients suffering from this rare skin disease. Psychosocial guidance is therefore desirable. Many of these patients also benefit from the use of antidepressants. Suicidal thoughts are common in these patients and close contact with a psychiatrist is strongly recommended.

Prognosis of severe lumps on skin and joint pain

Recurrent acne fulminans is extremely rare. The bone lesions usually disappear with treatment, but residual radiographic changes may still remain. Scarring and fibrosis may result from this acute inflammatory process.

read more

• Acne medicamentosa: Skin lesions caused by the use of medications
• Acne: Skin condition with inflammation of sebaceous glands
• Acne scars (boxcar scars): Treatment of scar formation
• Acne (pimples) on breasts: Causes & treatment of breast acne
• Acne conglobata: Lumps and pustules on the face, trunk and back