Journal Article

Rapid remission through multimodal therapy in a severe form of PASH Syndrome: A case report

Lohmer, Caspar Bernhard, von Stebut-Marx, Jennifer, Rasche, Claudia, Wilhelm, Tabea, Schneider-Burrus, Sylke

2026

Overview

PASH syndrome is a rare autoinflammatory dermatosis characterized by the triad of pyoderma gangrenosum (PG), acne conglobata, and hidradenitis suppurativa (HS), driven by innate immune dysregulation and IL-1–mediated neutrophilic inflammation. We report a 38-year-old woman presenting with severe HS (Hurley stage III), chronic nodulocystic facial acne, and PG-like ulcers, consistent with PASH syndrome. Her condition was marked by debilitating pain (VAS 8/10), malodorous discharge, and extensive ulceration, necessitating examination under general anesthesia. Previous therapies, including antibiotics and adalimumab, had failed. Surgical intervention involved fistula excision, wound debridement, and drainage. Histopathology confirmed neutrophilic inflammation without systemic disease. A combination of IV ertapenem, high-dose corticosteroids, and oral cyclosporine A led to rapid clinical improvement: pain resolution, mobility recovery, and complete healing of lesions within eight weeks. This case highlights the importance of early recognition of PASH through its cutaneous triad. Given the absence of standardized treatment guidelines, a multimodal, individualized approach, incorporating surgery, antimicrobial therapy, and immunosuppression is crucial. Our findings support the effectiveness of cyclosporine A and corticosteroids in achieving remission and underscore the need for multidisciplinary care in managing this complex dermatosis.