What Is Acne Fulminans?
Acne Fulminans Is a Very Rare and Extremely Severe Form of Acne
Acne fulminans is an extremely rare, severe form of acne that presents numerous painful nodules and/or cysts covering large areas of the skin along with symptoms that affect the whole body, such as fever, fatigue, and muscle pain. Notably, nodules and cysts in acne fulminans fill with blood and rapidly turn into open wounds that often leave massive, disfiguring scars. The disease debuts explosively and almost always affects young white males. The treatment of choice is a combination of oral corticosteroids and isotretinoin, often known by its brand name Accutane®.
Acne fulminans is an extremely rare, severe form of acne with an explosive onset that normally affects 13 - 22-year-old white males, and comes with systemic symptoms (symptoms that affect the whole body) like fever, fatigue, and pain in the muscles and joints.1,2 Due to its severity, acne fulminans often leaves massive, disfiguring scars. This type of acne falls under the umbrella term nodulocystic acne, which is used to refer to any type of acne that features, but is not necessarily limited to, large, red, painful acne lesions called nodules and/or cysts. The nodules and cysts in acne fulminans are unique in that they fill with blood and rapidly turn into open wounds called ulcers.
Acne fulminans is extremely rare. Only about 100 cases have been documented so far.1 The disease usually affects white males who are 13 - 22 years of age who have regular acne at first. On a few occasions, acne fulminans has been diagnosed in females.
Nobody knows what causes acne fulminans, but evidence suggests that the following three factors play a role.
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- Increased sensitivity to bacteria
- Elevated hormones
Increased sensitivity to bacteria
Propionibacterium acnes (P. acnes), the bacteria associated with acne, lives in everyone's skin. Some researchers hypothesize that the immune system in people with acne fulminans reacts to the presence of the bacteria even more so than in regular acne.1
Researchers suspect there is a gene responsible for predisposition to acne fulminans, which means that if a person's parents suffered from acne fulminans, that person's chances of getting it are much higher. Medical literature describes four cases in which identical twins developed acne fulminans with very similar symptoms.1
Medical professionals suspect that androgens (male sex hormones present in both males and females) contribute to the disease, particularly because androgens peak in puberty and young adolescent males get affected most often.1 Increased dehydroepiandrosterone sulfate (DHEAS) and testosterone (both male sex hormones) are a frequent finding in patients with nodulocystic acne, but no major studies have been performed to determine if that is also true for acne conglobata.
Acne fulminans usually occurs in individuals who have had mild to moderate acne for some time. The disease typically debuts in the early or middle teen years and almost never after the early twenties. Acne fulminans usually leaves massive, disfiguring scars.
The onset of the disease is very abrupt. Typically, a person has just average, regular acne for some time, usually for a year or two, and then numerous nodules and cysts suddenly begin to emerge on large areas of the skin on the upper chest, back, neck, and face.3,4 Less severe acne lesions like whiteheads, blackheads, papules, and pustules might be present as well, but they are never a dominant feature.
Systemic symptoms, including fever, fatigue, and/or joint and muscle pain, are always a part of the disease.1-4
Nodules and cysts: Nodules and/or cysts in acne fulminans are plentiful, and the neighboring lesions tend to merge with each other, forming extra-large lesions. Both nodules and cysts are over 5mm 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. Unlike in other forms of severe, nodulocystic acne, nodules and cysts in acne fulminans fill with blood and rapidly turn into painful ulcers (open wounds).1,3 Healing of ulcers can be extremely slow, often resulting in massive, disfiguring scars.
Scars: Scars in acne fulminans are often severe and widespread and can be atrophic (indented) or hypertrophic (raised above the skin).
Systemic symptoms: Acne fulminans is a systemic disease, which means that not only is the skin affected but the rest of the body as well. The most frequent systemic symptoms include:
- Muscle pain
- Tenderness, swelling, and pain in the joints
- Softening of the bones and increased risk of fractures
- Pain in the bones (occurs approximately in four out of ten patients)
- Enlargement of the liver and spleen
- Anemia (reduced red blood cell count)
- Leukocytosis (increased white blood cell count)
Acne fulminans vs. other severe forms of acne
One should be careful when differentiating acne fulminans from other forms of severe nodulocystic acne--acne conglobata and pyoderma faciale.
Acne conglobata: In acne conglobata, lesions tend to appear on the same areas of the face and body as in acne fulminans. Also, like in acne fulminans, neighboring nodules/cysts in acne conglobata often merge together and form extra-large, inflammatory lesions. What helps to distinguish between the two conditions is that nodules and cysts in acne fulminans fill with blood and rapidly turn into open wounds, while in acne conglobata they normally do not fill with blood and persist as raised lesions covered by the skin. In addition, acne fulminans patients commonly present with systemic symptoms, such as fever, joint pain, and weight loss, which are very uncommon in acne conglobata.
Pyoderma faciale: Pyoderma faciale is another form of nodulocystic acne, characterized by a sudden emergence of intense redness and large merging nodules and/or cysts on the face. The disease is relatively easy to differentiate from acne fulminans because pyoderma faciale occurs exclusively in females and affects the face only.
Because acne fulminans is so rare, very little clinical data is available for analysis. The only relatively large study that we identified suggested that a combination of oral corticosteroids (e.g., prednisolone) and isotretinoin is the preferred treatment.2,5 Isotretinoin is a highly controversial drug that must be taken only in close supervision with a doctor and is associated with numerous hazardous health effects. Only the severity of the disease justifies the use of this treatment option.
Oral corticosteroids and isotretinoin
Oral corticosteroids taken in combination with isotretinoin are the mainstay of treatment. Usually, treatment starts with a corticosteroid for the first six weeks. Isotretinoin is incorporated three to six weeks after corticosteroid treatment has begun, so the treatments sometimes overlap.1,5 In total, the treatment course lasts approximately six months. Corticosteroids work well on systemic symptoms and also reduce acne, while isotretinoin is necessary to achieve a long-lasting remission.
Clinical trials on the effectiveness of oral corticosteroids plus isotretinoin in the treatment of acne fulminans
A study published in the British Journal of Dermatology in 1999 reported that starting treatment with oral prednisolone and adding isotretinoin later in the course was the most effective treatment regimen for acne fulminans. Giving prednisolone first and adding isotretinoin a few weeks later was better than the converse because corticosteroids worked on systemic symptoms quickly.
Despite being highly effective in treating severe acne, isotretinoin often comes with a long list of side effects and, sometimes, even severe, toxic reactions. This is why it is approved for use only for severe, nodulocystic acne.
The most frequent side effects include:
- Inflammation of lips (90%)
- Dryness of skin and mucous membranes (80%)
- Pink eye (40%)
- Itching (40%)
- Gastrointestinal symptoms, such as nausea, vomiting, and abdominal pain (20%)
- Muscle and/or joint pain (16%)
- Increased sensitivity to light (10%)
Toxic reactions: Possible toxic reactions associated with the administration of isotretinoin might include inflammatory bowel disease and increased intracranial (in the head) pressure that causes moderate to severe headaches and visual impairments, such as blurred and double vision, difficulty seeing to the side, and brief episodes of blindness.6 The incidence of these events is unknown, but they are rather uncommon.
Adverse effect on the fetus: EXTREME CAUTION! Isotretinoin is contraindicated in pregnancy due to its severe, adverse effects on the fetus.6 In fact, it is the No.1 birth defect - causing medication on the market. Birth defects are often very severe, including death. Therefore, females are urged to obtain two (2) negative pregnancy tests prior to starting therapy. Additionally, women must use two (2) forms of contraception for 30 days both before and after treatment with isotretinoin.
Expand to see more side effects of isotretinoin and oral corticosteroid
Data on alternative treatment modalities for acne fulminans is limited and comes from scant case reports. On several occasions, prednisolone was used in combination with one or another of the following drugs.1,5,7
- Ciclosporin (regulates immune response)
- Infliximab (regulates immune response)
- Dapsone (anti-inflammatory)
- Antibiotics (erythromycin, tetracycline, or minocycline)
The researchers concluded that a combination of prednisolone and erythromycin is a viable treatment option during the early stages of acne fulminans and cystic acne, but isotretinoin should be added to establish long-term remission. As antibiotics in general are not effective, they should be employed as combination therapies.
Prednisolone combined with erythromycin was given to 6 patients with cystic acne. The treatment responses were compared to those in 6 patients with cystic acne receiving isotretinoin and erythromycin and also to those in 3 patients with acne fulminans treated with prednisolone and erythromycin. During the first 4 weeks cystic acne showed a clear improvement in 5 out of 6 patients in both treatment groups. A similar improvement occurred in all 3 patients with acne fulminans. When corticosteroid was stopped, 2 out of 5 patients with cystic acne had a relapse and needed isotretinoin for complete control. In the isotretinoin-treated group, one patient with cystic acne needed prednisolone because the acne worsened to an ulcerative form… The present results show that prednisolone combined with erythromycin is an effective treatment during the early stages of cystic and [acne accompanied by the symptoms of a fever], but isotretinoin is needed for long-term control.7
Acne fulminans is an extremely rare and unusual form of nodulocystic acne with a sudden onset and systemic symptoms that include, but are not limited to, fever, fatigue, and/or pain in the muscles and joints. Nodules and cysts in acne fulminans fill with blood and rapidly turn into open wounds that heal very slowly and usually leave massive, disfiguring scars. The disease almost always affects young white males. Clinical evidence shows that a combination of oral corticosteroids (prednisolone) and isotretinoin is the most effective treatment option. However, only a doctor can determine the optimal treatment course for each individual case.
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- Zaba, R. Acne Fulminans. Emedicine.medscape.com (2016). at http://emedicine.medscape.com/article/1072815-overview#showall
- Habif, T. Clinical Dermatology: A Color Guide to Diagnosis and Therapy (6th Edition). 231 - 233 (Saunders, 2015).
- Jansen, T. & Plewig, G. Acne fulminans. Int J Dermatol 37, 254 - 257 (1998).
- Karvonen, S. Acne fulminans: Report of clinical findings and treatment of twenty-four patients. J Am Acad Dermatol 28, 572 - 579 (1993)
- Seukeran, D. C. & Cunliffe, W. J. The treatment of acne fulminans: a review of 25 cases. Br J Dermatol 141, 307 - 309 (1999)
- Hodgson, B. & Kizior, R. Saunders nursing drug handbook 2012. (W. B. Saunders Co., 2014)
- Karvonen, S. Systemic corticosteroid and isotretinoin treatment in cystic acne. Acta Derm Venereol 73, 452 - 455 (1993).