Introduction:
Acne phlegmonosa is a severe inflammatory form of acne characterized by deep nodules, abscesses, and extensive scarring. While traditionally considered a dermatologic disorder, growing evidence suggests that in some patients it may represent a cutaneous manifestation of systemic autoinflammatory disease. Recent advances in immunology and genetics have linked severe acne to PAPA-spectrum syndromes, including PAPA, PASH, PAPASH, and PASS, which are characterized by dysregulated innate immunity and excessive production of proinflammatory cytokines such as IL-1β, TNF-α, and IL-17. Recognition of this autoinflammatory phenotype is important because it may influence both diagnostic evaluation and therapeutic decision-making, particularly regarding the use of targeted biologic therapies.

Aim:
The aim of this manuscript is to review current knowledge on the pathogenesis, clinical features, diagnosis, and treatment of acne with an autoinflammatory phenotype. Particular emphasis is placed on the role of inflammasome dysregulation, associated autoinflammatory syndromes, and emerging targeted biologic therapies.

Material and methods:
The literature review was conducted using the PubMed, Scopus, and Web of Science databases, covering all available publications up to March 2026.

Conclusions:
The coexistence of acne phlegmonosa with systemic manifestations such as fever, cutaneous ulcerations, and arthritis should prompt consideration of an underlying autoinflammatory syndrome. Awareness of entities such as PAPA, PASH, PAPASH, and PASS is therefore crucial. Timely identification of these conditions allows for the early introduction of systemic immunomodulatory therapy, extending beyond standard dermatologic management of acne.