Introduction: Eosinophilic pustular folliculitis (EPF; Ofuji disease) is a rare, usually chronic relapsing inflammatory skin disorder characterized by pruritic follicular papules and pustules and an eosinophil-rich inflammatory infiltrate. The main clinical variants are classic EPF, immunosuppression-associated EPF (including HIV-associated disease), and infantile EPF.

Aim: To summarize the clinical spectrum, proposed pathogenetic mechanisms, diagnostic features, and current therapeutic approaches to EPF.

Materials and Methods: A literature search was conducted in PubMed/MEDLINE, Scopus, and Web of Science for publications indexed between July 2000 and July 2026. Original research articles, systematic reviews, retrospective studies, and relevant clinical case reports were considered. Particular emphasis was placed on recent advances in understanding disease pathogenesis, diagnostic approaches, therapeutic strategies, and emerging treatment options. The identified literature was critically evaluated and synthesized to provide an updated overview of current knowledge and EPF management.

Results: Diagnosis requires correlation of clinical and histopathological findings and exclusion of infectious and other follicular dermatoses. The pathogenesis is multifactorial, with the type 2 immune response, eosinophil recruitment, and the PGD2–PPARγ– CCL26 and IL-36 signaling pathways among the mechanisms under investigation. Treatment depends on the clinical variant, disease severity, age, and immune status. Indomethacin has the strongest supporting evidence among systemic treatments for classic EPF, whereas topical corticosteroids and management of the underlying condition are important in other variants. Alternative anti- inflammatory and targeted therapies have been reported for refractory cases; however, the available evidence is based predominantly on small case series and individual case reports.

Conclusion: Given the clinical and pathogenetic heterogeneity of EPF, an individualized diagnostic and therapeutic approach, careful clinicopathological correlation, and appropriate follow-up are essential. Further studies are needed to establish the efficacy and safety of emerging targeted therapies.