Acne Fulminans


Article Author:
Patrick Zito


Article Editor:
Talel Badri


Editors In Chief:
Sisira Reddy
Joseph Nahas
Chokkalingam Siva


Managing Editors:
Avais Raja
Orawan Chaigasame
Khalid Alsayouri
Kyle Blair
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Abbey Smiley
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beenish Sohail
Hajira Basit
Phillip Hynes
Sandeep Sekhon


Updated:
12/2/2018 12:56:40 PM

Introduction

Acne fulminans or acne maligna is a rare skin disorder presenting as an acute, painful, ulcerating, and hemorrhagic clinical form of acne. It may or may not be associated with systemic symptoms such as fever and polyarthritis. Acne fulminans also may cause bone lesions and laboratory abnormalities. Acne fulminans often is confused with acne conglobata. The condition usually is resistant to the usual acne antibiotics.[1][2][3]

Etiology

Acne fulminans is a rare skin disorder, and hence its etiology is not well understood. Acne fulminans may be triggered by the use of high doses of isotretinoin when initiating treatment in patients with severe acne. Also, elevated levels testosterone may have a role in the pathogenesis. Anabolic steroids are known to increase levels of sebum and density of Propionibacterium acnes. Bodybuilders who use anabolic steroids develop acne fulminans within a very short time. It is believed that the density of P. acnes or related antigens trigger the immune system and lead to acne fulminans. There are others who believe the condition to be autoimmune mediated because circulating immune complexes have been observed in some patients with acne fulminans.[4][5]

Acne fulminans also has a genetic component, but the pattern of inheritance is not known. The skin disorder may be part of the synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome (SAPHO), pyogenic arthritis, pyoderma gangrenosum, and acne syndrome (PAPA), pyoderma gangrenosum, acne, and hidradenitis suppurativa syndrome (PASH), and pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa syndrome (PAPASH).

Epidemiology

Less than 200 cases are known. The disorder usually affects young males, mainly Caucasian, between 13 and 22 years of age. Most of these males have a prior history of acne. There also are isolated reports of acne fulminans in females. The condition has been reported globally, but its incidence seems to be declining.

History and Physical

Acne fulminans usually has a very sudden onset, and the acne often is ulcerating. Patients may complain of fever and pain in multiple joints. They usually provide a history that the conventional antibiotics used for treating acne did not work. Most patients mention that they did have acne in the past.

Acne fulminans is similar to acne conglobata with numerous inflammatory nodules on the trunk. The nodules often are painful to touch, ulcerated, hemorrhagic, and covered with crusts. Unlike acne conglobata, there are cysts and acutely inflamed lesions, but there are no polyporous comedones. Painful splenomegaly and erythema nodosum may be associated with systemic symptoms.

Patients with acne fulminans who have systemic symptoms may have a stooped posture due to bone and joint pain. The inflammatory arthralgia usually affects more than one joint, typically the knees, hips, and pelvis.

Evaluation

Because the disorder may be associated with systemic symptoms, the workup should include the following:

  • Complete blood cell count (CBC); may show elevated white blood cell (WBC) count with a large percentage of neutrophils, anemia of chronic disease, and/or leukemic-type reaction
  • Erythrocyte sedimentation rate (ESR) and C-reactive protein may be elevated
  • Liver function test
  • Human chorionic gonadotrophin (blood or urine) in female patients

Imaging

Because of bone and joint pain, imaging frequently is done. In the early stage, x-rays will only show reactive changes. About 50% of patients will demonstrate lytic lesions on a plain x-ray, and the bone scan may show multiple areas of increased uptake. The lesions are destructive and often resemble acute osteomyelitis. Cultures of these bony lesions are often negative.

Treatment / Management

The recommended treatment for acne fulminans is a combination of corticosteroids and isotretinoin. Oral corticosteroids should be started first at high doses (0.5 to 1 mg/kg/day) for at least 2 weeks (at least 4 weeks if systemic symptoms) until lesions heal. Isotretinoin is then started. The initial dose of isotretinoin should be 0.1 mg/kg/day in association with corticosteroids for 4 weeks. If no flare is observed, the same dose of isotretinoin should be maintained for 4 more weeks, and corticosteroid doses should be gradually tapered. Increasing isotretinoin doses should then be progressed to a minimum total dose of 120 mg/kg.[6][7][8][9]

In patients who remain adherent, relapses are rare. If a relapse does occur, a repeat cumulative dose of isotretinoin at 150 mg/kg is recommended. The treatment with isotretinoin lasts for many months as the initial dose is low.

A recent review suggests that the use of additional high-potency topical corticosteroids may result in a faster response. Once healing occurs, the lesions become smaller, less tender, and have a minimal inflammatory reaction. Scarring is common.

Acne fulminans does not respond to the traditional antibiotics used to treat the typical form of acne. Even when they are used, the response is slow and incomplete.

In the last few years, anecdotal reports have appeared after use of biologics, such as etanercept and infliximab, in patients who are resistant. The response to these agents is effective and rapid. However, it is not known if biologics will work in all patients. Several other targeted biologics also have been used to treat acne fulminans.

Other treatments

Pulsed dye laser has been used with moderate effectiveness, but adverse effects and pain remain an issue. Because the lesions are large, multiple treatments are required, and scarring and hypopigmentation are common complications.

Reports suggest that diaminodiphenyl sulfone use may help ease the symptoms of acne fulminans in patients with ulcerative colitis.

Combinations of prednisone with dapsone or cyclosporine also have been tried with success.

Finally, these patients are best managed by a dermatologist and an internist. Women of childbearing age should use great caution when using retinoids, as these agents are teratogenic. There is a registry of all patients who are prescribed retinoids. All women who are prescribed isotretinoin should avoid getting pregnant for at least one month after the drug is discontinued.

Enhancing Healthcare Team Outcomes

Acne fulminans is a devastating systemic disorder with severely disfiguring skin lesions. The primary care provider and nurse practitioner should know that this is a severe form of acne that should be referred to a dermatologist.  The majority of patients are affected negatively by the poor cosmesis. Their quality of life is altered, and they are not able to participate in sports or social activities. Many become withdrawn and isolated. Thus, it is vital to offer psychosocial counseling to them. Many of these patients will benefit from mental health treatment and the use of antidepressants. Suicidal ideation is very common in these patients and close follow up with a psychiatrist is highly recommended. The use of retinoids has not been shown to worsen the risk of depression or suicidal ideation.[10][11]

The patient also has to be educated by clinicians, nurses, and pharmacists on the skin disorder and avoidance of triggering agents. The skin should be kept clean and dry, smoking should be discontinued, and one should refrain from applying unproven remedies like oils and ointments. A team approach is most effective.


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Acne Fulminans - Questions

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Which feature do acne fulminans and acne conglobata share?



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Which of the following is not characteristic of acne fulminans?



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Acne fulminans is a rare, severe form of acne conglobata associated with systemic symptoms. It nearly always affects adolescent males. Which of the following would be expected?



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A patient presents with inflammatory and ulcerated painful nodular acne on the chest and back. He has a fluctuating fever and his joints are painful. He has malaise, loss of appetite, and weight loss. On exam, his liver and spleen are palpably enlarged. Which of the following would be expected?



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A patient presents with inflammatory and ulcerated nodular acne on the chest and back that is painful. He has a fluctuating fever and arthralgia. He also has malaise, loss of appetite, and weight loss. On exam, his liver and spleen are palpably enlarged. Which of the following would be expected?



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Which of the following dermatologic conditions may require a rheumatologist to assist in the diagnosis?



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Which of the following dermatologic conditions require a radiologist to assist in the diagnosis?



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A patient presents with inflammatory and ulcerated painful nodular acne on the chest and back. He has a fluctuating fever and his joints are painful. He has malaise, loss of appetite, and weight loss. On exam, his liver and spleen are palpably enlarged. Which of the following is the most appropriate initial treatment?



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Acne fulminans is a systemic syndrome of necrotizing acne and is frequently associated with involvement of which other organ system?



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A 33-year-old male bodybuilder presents to an urgent care facility with sudden onset of painful nodules on the trunk and pain in multiple joints. Detailed medical history reveals that he has been using anabolic steroids for the past two years. He also told the clinician that he was prescribed minocycline by a local health practitioner, but no avail. On physical examination, the nodules are painful to touch, ulcerated, hemorrhagic, and covered with crusts, but there are no polyporous comedones. A skin biopsy of the lesion was taken and sent for pathology evaluation. The cells of the specimen tissue stained violet on gram staining test. Which one of the following triggering agent is responsible for this disease?



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Which of the following can cause acne fulminans?



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A 16-year-old male presents to the clinic with complaints of sudden onset of fever, pain in multiple joints, and severe ulcerating acne. He tried to use clindamycin and benzoyl peroxide, but his acne has not responded. The exam reveals the presence of large painful ulcerated nodules over his trunk. There do not appear to be any comedones. He may have developed which of the following complications?



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A young patient presents to the clinic with complaints of sudden onset of fever, pain in multiple joints, and severe ulcerating acne. He tried to use clindamycin and benzoyl peroxide, but the acne has not responded. The exam reveals the presence of large painful ulcerated nodules over his trunk. There do not appear to be any comedones. Abdominal exam reveals marked tenderness in the left upper quadrant. What treatment would should the patient be started on?



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Acne Fulminans - References

References

Gualtieri B,Panduri S,Chiricozzi A,Romanelli M, Isotretinoin-triggered acne fulminans: a rare, disabling occurrence. Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia. 2018 Sep 18     [PubMed]
Gualtieri B,Tonini A,Panduri S,Chiricozzi A,Romanelli M, Acne fulminans associated with lymecycline intake: a case report. Clinical, cosmetic and investigational dermatology. 2018     [PubMed]
Li AW,Antaya RJ, Isotretinoin-induced acne fulminans without systemic symptoms with concurrent exuberant granulation tissue. Pediatric dermatology. 2018 Mar     [PubMed]
Proença NG, Acne fulminans. Anais brasileiros de dermatologia. 2017     [PubMed]
Massa AF,Burmeister L,Bass D,Zouboulis CC, Acne Fulminans: Treatment Experience from 26 Patients. Dermatology (Basel, Switzerland). 2017     [PubMed]
Walsh RK,Endicott AA,Shinkai K, Diagnosis and Treatment of Rosacea Fulminans: A Comprehensive Review. American journal of clinical dermatology. 2018 Feb     [PubMed]
Dessinioti C,Katsambas A, Difficult and rare forms of acne. Clinics in dermatology. 2017 Mar - Apr     [PubMed]
Siadat AH,Bostakian A,Abtahi-Naeini B,Shahbazi M, Successful Treatment of Facial Acne Fulminans: Antimicrobial Agents and Oral Prednisolone as Promising Regimes. Case reports in dermatological medicine. 2017     [PubMed]
Perez M,Navajas-Galimany L,Antunez-Lay A,Hasson A, When strength turns into disease: acne fulminans in a bodybuilder. Anais brasileiros de dermatologia. 2016 Sep-Oct     [PubMed]
Brănişteanu DE,Cotrutz CE,Luca MC,Molodoi DA,Stoica LE,Ianoşi SL,Cianga CM,Brănişteanu DC, Morphopathological stigmata in acne fulminans. Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie. 2015     [PubMed]
Kraus SL,Emmert S,Schön MP,Haenssle HA, The dark side of beauty: acne fulminans induced by anabolic steroids in a male bodybuilder. Archives of dermatology. 2012 Oct     [PubMed]

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