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A 14-year-old male with acute acne flare
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A 14-year-old boy presents for evaluation of flaring acne. The patient has had acne on his face, chest and back for approximately 6 months. He notes his acne was initially mild, but during the past month has dramatically flared.
The patient has been taking oral doxycycline and using topical adapalene/benzoyl peroxide for 30 days with no improvement. Upon review of systems, he notes a fever and joint pain. On physical exam, severe scattered cystic and inflammatory lesions are seen, along with several areas of hemorrhagic crusting and extensive scarring.
Shehla Admani
Andrew C. Krakowski
The patient cannot recall any potential triggers for his acne flare. He denies illicit drug use; he is on the varsity baseball team but denies experimenting with any performance-enhancing substances. He is otherwise in good health and is not taking any other medications. He has no personal or family history of depression or inflammatory bowel disease.
What changes should be made to the patient’s treatment regimen?
- Keep it the same, 30 days is not sufficient time to see any improvement.
- Switch the patient to a different oral antibiotic because he is likely having a drug reaction.
- The patient has scarring, so start isotretinoin immediately.
- Begin a course of oral prednisone, being careful to taper slowly to avoid rebound inflammation.
- Inject the individual cysts with intralesional steroid.
Diagnosis: Acne fulminans (AF) is the most severe form of cystic acne and is one of the most scarring acute dermatologic conditions seen in young patients. AF is rare and primarily affects adolescent white males. Patients with AF typically have mild to moderate papulopustular or cystic acne for 1 year before the onset of their acute symptoms. The etiology of AF is unclear; however, immunological reactions to Propionibacterium acnes, altered neutrophil function and genetic factors have all been implicated in the pathogenesis of this disease. In addition, isotretinoin has been suspected to increase fragility of the pilosebaceous duct, leading to extensive contact between P. acnes antigens and the immune system, which may precipitate AF.
The classic presentation of AF consists of sudden onset of fever, severe acne and polyarthritis. The acne lesions are highly inflammatory and can have ulceration and hemorrhagic crusting. They are typically more severe on the back and chest than the face and can be very tender, making it difficult for the patient to lie on his or her back. Arthralgias and effusions can be seen most commonly in large joints such as sacroiliac, iliac and knee joints, making ambulation difficult.
Left cheek with inflammatory lesions and extensive scarring.
Other more rare manifestations of AF include erythema nodosum, aseptic bone osteolysis, hepatosplenomegaly and myositis. There are no characteristic laboratory abnormalities; however, patients can have a leukocytosis and elevated erythrocyte sedimentation rate. Bacterial cultures from blood, joint fluid and skin are usually sterile.
The treatment of AF can be challenging, and high rates of relapse can be seen if caution is not used when tapering anti-inflammatory steroids. Systemic steroids are the drug of choice because they can quickly improve the skin lesions, reduce fever and decrease musculoskeletal symptoms. Providers should also be aware of the potential effects of a prolonged course of system corticosteroids and take necessary precautions such as gastric ulcer prevention. If the steroid dose is lowered or discontinued too quickly, symptoms can recur and typically consist of worsening of the arthralgias, fever, or even the skin lesions themselves. To avoid relapse, steroid therapy duration can be at least 2 to 4 months.
Back and shoulder with scattered cystic and inflammatory lesions, scarring and few lesions with hemorrhagic crust.
Although isotretinoin has been implicated as a potential precipitant of AF, after the acute inflammatory phase of AF is controlled (with approximately 4 weeks of corticosteroid treatment), the addition of isotretinoin can be beneficial. Duration of treatment depends upon individual response and usually should not be less than 3 to 5 months. In cases where isotretinoin is not available, dapsone, infliximab (Remicade, Janssen Biotech) and cyclosporine have all been used successfully in treating patients with AF.
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For more information:
Shehla Admani, MD, is a Clinical Research Fellow in Pediatric Dermatology at Rady Children’s Hospital, San Diego. Andrew C. Krakowski, MD, is an attending physician at Rady Children’s Hospital, San Diego.
Disclosure: Admani and Krakowski report no relevant financial disclosures.