[Severe acne in autoinflammatory diseases].

BACKGROUND

In recent years, acne has been described as a symptom of autoinflammatory diseases, such as PASH (pyoderma gangrenosum - PG, acne and suppurative hidradenitis - SH) and PAPASH (PG, acne, pyogenic arthritis, and SH). The pathogenesis of autoinflammatory diseases is not fully understood; however, based on the possible involvement of IL-1β, the recombinant human interleukin-1 receptor antagonist anakinra has been used in the treatment of certain autoinflammatory diseases.

METHODS

We describe two patients with severe acne and associated symptoms which led to the diagnosis PAPASH and PASH syndrome and who were treated with anakinra.

RESULTS

In the patient with PASH syndrome, inhibition of inflammation and almost complete healing of ulcers was observed. In the patient with PAPASH syndrome, partial response was achieved.

CONCLUSION

The therapeutic effect of anakinra in PASH syndrome and partly in PAPASH syndrome indicates an involvement of IL-1β in acne-associated autoinflammatory diseases.