54337 Full-body Multifocal Pyoderma Gangrenosum in the setting of PASH Syndrome
Recommended Citation
Oska S, Konda S, Matthews N. 54337 Full-body Multifocal Pyoderma Gangrenosum in the setting of PASH Syndrome. J Am Acad Dermatol 2024; 91(3):AB47.
Document Type
Conference Proceeding
Publication Date
9-1-2024
Publication Title
J Am Acad Dermatol
Abstract
Presentation: A 26-year-old male with history of hidradenitis suppurativa (HS) of the groin presented with a two-year worsening rash. He was cachectic on examination with innumerable tender ulcerations with violaceous borders and purulent drainage on the scalp, face, trunk, and all four proximal extremities. He also exhibited mild facial acne vulgaris and sinus tracts with multiple scars in the bilateral inguinal folds consistent with his known HS. He denied personal or family history of autoinflammatory, autoimmune, or inflammatory bowel disease (IBD). He denied any fevers, chills, arthralgias, abdominal pain, or changes in bowel movements. Differential diagnosis included pyoderma gangrenosum (PG), PASH (PG, acne, and HS) syndrome, pemphigus vegetans, and infection. Course and Therapy: Punch biopsy of a chest lesion showed ulcer with granulation tissue and a neutrophilic-rich dermal infiltrate. Tissue culture was negative. Work-up was negative for inflammatory arthritis, IBD, connective tissue disease, or malignancy. He was diagnosed with PASH syndrome. He underwent treatment with high-dose corticosteroids, doxycycline, and ultimately infliximab with significant improvement of the ulcerations. Prior to treatment, he was non-ambulatory due to pain. He was able to ambulate after starting infliximab. Discussion: Our patient exhibited a strikingly diffuse presentation of PG in the setting of suspected PASH syndrome. PASH and other autoinflammatory syndromes are characterized by sterile neutrophil-dense inflammation. Mutations affecting proteins of the inflammasome complex are thought to play a role in its pathogenesis, leading to over-expression of IL-1 and tumor necrosis factor (TNF) alpha. Thus, TNF-alpha inhibitors and IL-1 inhibitors are often utilized for treatment.
Volume
91
Issue
3
First Page
AB47
