FROM journal of the american academy of dermatology

The incidence of acne fulminans may be decreasing but the isotretinoin-induced form is on the rise, say the authors of new guidelines on this severe variant of inflammatory acne.

Writing in the July issue of the Journal of the American Academy of Dermatology, Tanya Greywal, MD, from the University of California, San Diego, and coauthors, presented guidelines that aimed to better characterize this relatively rare condition and provide guidance on classification, treatment, and prevention.

Acne fulminans presents with rapid onset of painful erosions and hemorrhagic crusts – usually on the trunk – that can lead to severe, disfiguring scars. A more extreme form of the disorder also features systemic inflammation including fever, arthralgia, osteolytic bony lesions, and can even require hospitalization.

It is most commonly seen in white adolescent males, many of whom have a prior history of acne (J Am Acad Dermatol. 2017;77:109-17).

While alterations in innate immunity, autoimmunity, adaptive immunity, and autoinflammation have all been suggested as playing a role in the pathogenesis of acne fulminans, it can also be associated with isotretinoin use.

The overall incidence of the disorder has been decreasing over the last decade, which some suggest may be because the disease is being recognized and treated earlier. However, isotretinoin-induced acne fulminans is on the rise, perhaps because of more widespread use of the drug.

Acne fulminans previously has been described with a range of terms, including acne maligna, acute febrile ulcerative acne conglobota, and pseudo-acne fulminans, which the authors said has led to some confusion.

However, the expert panel behind the guidelines proposed that acne fulminans should be classified as being either with or without systemic symptoms, and either isotretinoin-induced or not.

They also recognized a range of associated disorders, including SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis), PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum, and acne), and PASH (pyoderma gangrenosum, acne, and hidradenitis suppurativa).

In the absence of large-scale randomized controlled trials of treatments for acne fulminans, the authors said case series and individual reports supported the use of systemic corticosteroids in combination with isotretinoin when treating all forms of the disorder.

The expert group recommended starting patients on prednisone 0.5 to 1 mg/kg per day as monotherapy for at least 4 weeks for acne fulminans with systemic symptoms, and for at least 2 weeks in the absence of systemic symptoms.

They proposed a typical isotretinoin cumulative goal dose of 120-150 mg/kg, starting at a lower dose and gradually increasing, and overlapping with prednisone.

Case studies suggest that tetracyclines are minimally effective against acne fulminans, but the authors said there was a need for studies to examine whether the use of antibiotics overlapping with isotretinoin might reduce the development of isotretinoin-induced acne fulminans.

No funding or conflicts of interest were declared.