Acne fulminans (AF), also known as acne maligna, was originally described as acute febrile ulcerative acne conglobata (AC).
The primary features of this disease include sudden onset, severe and often ulcerating acne, fever, polyarthritis, and failure to respond to antibacterial therapy; the response to debridement in combination with steroid therapy is good. It can be the dermatologic manifestation of the synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome. Acne fulminans is a syndrome of fulminant, necrotizing acne associated with bone lesions, constitutional symptoms, and laboratory abnormalities. |
| Symptoms |
- Abrupt onset
- Inflammatory and ulcerated nodular acne on chest and back
- Severe acne scarring
- Fluctuating fever
- Painful joints
- Malaise (ie. the patient feels unwell)
- Loss of appetite and weight loss
- Raised white blood cell count.
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| Treatment |
Treatment should be sought immediately in order to avoid hospitalization. If not treated, hospitalization for an extended period of time (usually two weeks) is likely.Corticosteroids or non-steroidal anti-inflammatory medications may be given to reduce inflammation. Attacks of acne fulminans may recur, and the patient may develop acne that requires long-term treatment with isotretinoin.
Topical acne medications are unhelpful. |
| Causes |
Acne fulminansis an uncommon, immunologically induced, systemic disease in which the triggering antigen is believed to be from Propionibacterium acnes. Some authors note that elevated blood levels of testosterone may play an important role in the pathogenesis of acne fulminans.Genetic factors may play an important role in some patients. |
| Tests & diagnosis |
Findings in patients with acne fulminans include the following:
- Leukocytosis (characteristic finding)
- Increased percentage of polymorphonuclear leukocytes
- Anemia
- Leukemic-type reaction
- Elevated erythrocyte sedimentation rate
- Circulating immune complexes
- Proteinuria
Blood cultures are universally sterile.
Imaging Studies
Bone involvement is common.
- Approximately 50% of patients have lytic bone lesions demonstrated on radiographs, and 70% of patients show increased uptake using technetium scintillography ("hot spots").
- Destructive lesions resembling osteomyelitis are demonstrated on radiographs in 25% of patients.
- Multifocal osteolytic cysts may be evident as tender bones and can be detected as hot spots by technetium scintillography.
Histologic Findings
Analysis of biopsy specimens of the bony lesions shows reactive changes only. |
| Prognosis |
- Recurrent AF is extremely rare.
- Bone lesions typically resolve with treatment, but residual radiographic changes, such as sclerosis and hyperostosis, may remain.
- Scarring and fibrosis may result from this acute inflammatory process.
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| Complications |
Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome. |
