Acne fulminans in Adult
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Synopsis

Acne fulminans is a rare, highly inflammatory, immunologically induced form of acne that occurs mainly in male patients between the ages of 13 and 22 years. Individuals of Northern European descent are predominantly affected. Individuals of East Asian descent may experience a milder form of acne fulminans.
While the pathogenesis of acne fulminans remains to be fully elucidated, the main inciting antigen is believed to be from Cutibacterium acnes (formerly known as Propionibacterium acnes). Testosterone exacerbates the process by increasing sebum excretion and the population density of C acnes.
Risk factors for acne fulminans include chronic severe acne (mean duration of 2 years), isotretinoin use (usually in high doses), a positive family history, high testosterone levels, and history of anabolic steroid use.
In acne fulminans without systemic symptoms (AF-WOSS), patients present with a sudden onset of large inflammatory cysts, nodules, papules, pustules, and abscesses. In acne fulminans with systemic symptoms (AF-SS), patients are febrile with systemic complaints, which may include polyarthralgia, myalgias, malaise, anorexia, and weight loss. Splenic tenderness, erythema nodosum, and bone pain from aseptic osteolysis have been reported.
When isotretinoin treatment induces acne fulminans in individuals with severe acne, this is known as isotretinoin-induced acne fulminans without systemic symptoms (IIAF-WOSS) since systemic symptoms are typically absent. In rare cases, systemic symptoms accompany, and the diagnosis is known as isotretinoin-induced acne fulminans with systemic symptoms (IIAF-SS).
Acne fulminans can be the dermatologic manifestation of SAPHO (synovitis, pustulosis, hyperostosis, and osteitis), PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne), PASH (pyoderma gangrenosum, acne, and suppurative hidradenitis), and PAPASH (pyogenic arthritis, pyoderma gangrenosum, acne, and suppurative hidradenitis) syndromes.
While the pathogenesis of acne fulminans remains to be fully elucidated, the main inciting antigen is believed to be from Cutibacterium acnes (formerly known as Propionibacterium acnes). Testosterone exacerbates the process by increasing sebum excretion and the population density of C acnes.
Risk factors for acne fulminans include chronic severe acne (mean duration of 2 years), isotretinoin use (usually in high doses), a positive family history, high testosterone levels, and history of anabolic steroid use.
In acne fulminans without systemic symptoms (AF-WOSS), patients present with a sudden onset of large inflammatory cysts, nodules, papules, pustules, and abscesses. In acne fulminans with systemic symptoms (AF-SS), patients are febrile with systemic complaints, which may include polyarthralgia, myalgias, malaise, anorexia, and weight loss. Splenic tenderness, erythema nodosum, and bone pain from aseptic osteolysis have been reported.
When isotretinoin treatment induces acne fulminans in individuals with severe acne, this is known as isotretinoin-induced acne fulminans without systemic symptoms (IIAF-WOSS) since systemic symptoms are typically absent. In rare cases, systemic symptoms accompany, and the diagnosis is known as isotretinoin-induced acne fulminans with systemic symptoms (IIAF-SS).
Acne fulminans can be the dermatologic manifestation of SAPHO (synovitis, pustulosis, hyperostosis, and osteitis), PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne), PASH (pyoderma gangrenosum, acne, and suppurative hidradenitis), and PAPASH (pyogenic arthritis, pyoderma gangrenosum, acne, and suppurative hidradenitis) syndromes.
Codes
ICD10CM:
L70.8 – Other acne
SNOMEDCT:
4659007 – Acne fulminans
L70.8 – Other acne
SNOMEDCT:
4659007 – Acne fulminans
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Acne conglobata lacks many of the systemic manifestations of acne fulminans, and onset is typically at a later age. Acne conglobata exhibits polyporous comedones and noninflammatory cysts.
- Acne vulgaris
- Severe cystic acne (see acne vulgaris)
- Chloracne and other forms of occupational acne
- Staphylococcal or gram-negative folliculitis
- Rosacea
- Hidradenitis suppurativa
- Bromoderma
- Iododerma
- Rosacea fulminans
- Steatocystoma multiplex may become infected but usually does not display the systemic findings.
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
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References
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Last Reviewed:09/12/2021
Last Updated:09/19/2021
Last Updated:09/19/2021