Pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome is a rare autosomal dominant hereditary autoinflammatory disease caused by PSTPIP1 gene variants and belongs to the PSTPIP1-associated inflammatory diseases (PAIDs). Its core clinical manifestations include recurrent pyogenic arthritis, pyoderma gangrenosum, and severe acne with onset in childhood or adolescence. Some patients may also present with multisystem involvement, such as inflammatory bowel disease and scleritis. Inflammation markers, such as CRP and ESR, are often significantly elevated. Treatment mainly involves targeted inhibition of inflammatory pathways, such as IL-1 inhibitors and TNF-α inhibitors. In this article, we report a Chinese patient with PAPA syndrome with disease onset at 13 years of age, whose main manifestations were pyoderma gangrenosum and acne. Genetic testing revealed a de novo PSTPIP1 gene variant (c.748G>A, p.Glu250Lys). We also reviewed recent literature on PAPA syndrome, summarizing its clinical manifestations, diagnosis, and treatment to enhance physicians' understanding of the condition.

PurposeTo characterize the spatiotemporal patterns of vascular remodeling following pterygium excision with amniotic membrane transplantation (AMT) using optical coherence tomography angiography (OCTA).MethodsThis observational study evaluated 43 eyes undergoing AMT. Serial OCTA imaging was performed over a 12-week postoperative period to quantify vascular dynamics in superficial and deep conjunctival layers. Quantitative parameters, including vessel density (VD), vessel length density (VLD), and vessel diameter index (VDI), were analyzed using automated image processing techniques.ResultsEarly assessment at Week 1 showed minimal vascular in-growth. By Week 4, progressive remodeling was evident, primarily driven by deep-layer anastomoses between host tissue and the graft area. By Week 12, a structured vascular network emerged; however, superficial perfusion remained significantly reduced compared to baseline. While deep-layer VD demonstrated substantial recovery, VDI remained statistically stable. Notably, OCTA identified persistent flow voids and heterogeneous perfusion, suggesting irregular host-derived vascular integration beneath the graft.ConclusionPost-AMT healing is mediated predominantly by deep-layer episcleral vascular remodeling. OCTA serves as a sensitive, non-invasive tool for monitoring subclinical vascular integration, offering potential biomarkers for surgical success and early pterygium recurrence.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune disorders that cause inflammation and necrosis of small- to medium-sized blood vessels. It most commonly affects the kidneys and lungs, leading to complications such as rapidly progressive glomerulonephritis and pulmonary hemorrhage. (1) Ocular involvement occurs in up to 50% of patients with AAV, most commonly presenting as scleritis, episcleritis, or orbital inflammatory disease (also known as orbital pseudotumor). Other ocular findings may include keratitis, uveitis, or retinal vasculitis. (2)(3) Eyelid necrosis with destructive orbital inflammation, however, is extremely rare and poses a significant diagnostic challenge because its presentation can mimic infections, malignancies, or other inflammatory orbital conditions. Prompt recognition is critical to prevent irreversible tissue damage and vision loss. (4) We report the case of a 74-year-old Hispanic woman with multiple vascular risk factors who presented with recurrent pustules of the right upper eyelid that progressed to full-thickness eyelid necrosis, medial orbital mass effect, and corneal compromise. Initial management was directed toward presumed orbital cellulitis; however, orbital imaging revealed a destructive extraconal mass with mass effect and associated venous stasis. Sequential biopsies demonstrated extensive necrosis with histiocytoid granulomatous inflammation and focal features of chronic vasculitis. Negative stains and cultures excluded infectious and neoplastic causes. After thorough evaluation and exclusion of alternative etiologies, the findings were most consistent with ANCA-negative ANCA-associated vasculitis (AAV). The patient was treated with high-dose intravenous corticosteroids and rituximab induction, resulting in stabilization of orbital disease and preservation of vision. This case highlights the potential for destructive orbital vasculitis to masquerade as infection or malignancy and underscores the importance of considering AAV in atypical orbital presentations. Prompt recognition and initiation of immunosuppressive therapy are critical to prevent irreversible morbidity.

Syphilis can present with a wide range of ocular manifestations; however, scleritis is exceedingly rare. Although various infectious and noninfectious causes of scleritis following strabismus surgery have been documented, syphilitic scleritis has not been previously reported in this context. We present the case of a 77-year-old man who underwent bilateral horizontal strabismus surgery complicated by postoperative necrotizing scleritis. Initial postoperative treatment with antibiotics and oral corticosteroids did not lead to significant improvement. The patient underwent an extensive rheumatologic and infectious workup and was treated for latent syphilis, resulting in a marked improvement and quiescence of his scleritis.