An unusual case of cutaneous manifestations of p-anca vasculitis mimicking stevens- johnson syndrome

SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters 2 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 12:00 pm - 12:45 pm INTRODUCTION: Dermatological findings are very common in systemic autoimmune vasculitis and more likely with anti-neutrophilic cytoplasmic antibodies (ANCA) associated vasculitis. The skin manifestations vary based on the course of disease, time of onset, and the type of vasculitis. The presence of cutaneous features like palpable purpura, ulcers, infarction, or nodules along with other systemic finding are clues to the diagnosis of vasculitis. The presentation of ANCA associated vasculitis as Stevens-Johnson Syndrome (SJS) is very uncommon. CASE PRESENTATION: A 37-year-old male with a past medical history of recurrent ear infections, resistant hypertension, and smoking presented to the emergency department with a three day history of diffuse body rash, facial lesions, hematuria, and drainage from the bodily orifices. The rash appeared to be maculopapular, erythematous, and painful. It initially only involved the genital region before spreading to the face and becoming diffuse associated with sloughing of the skin. Labs revealed leukopenia with eosinophilia. The Nikolsky sign was positive which was concerning for SJS as the patient had recently been prescribed antibiotics. Given this, he was admitted to the Burn Unit. The chest X-ray and CT of the chest were consistent with bullous emphysema. An autoimmune workup revealed elevated ANA titers, a positive p-ANCA, as well as myeloxperoxidase specificity. The pathological analysis of the biopsy sample from the cutaneous lesions identified an abundant, essentially perivascular polymorphous inflammatory infiltrate with a marked leukocytoclastic pattern associated with vasculitis. He was diagnosed with p-ANCA vasculitis and started on high dose corticosteroids and immunosuppressants. Lastly, a renal biopsy has been performed and was consistent with glomerulonephritis due p-ANCA vasculitis. His cutaneous lesions were improving upon treatment. DISCUSSION: Although cutaneous involvement is not a common feature of p-ANCA vasculitis, a few case reports have described similar presentations. In a case report published in the Indian Journal of Dermatology, p-ANCA vasculitis presented with a painful, erythematous rash having the features of both urticarial vasculitis and SJS. Another case report published in the Journal of the European Academy of Dermatology and Venereology, described a 52-year-old male with p-ANCA vasculitis who presented with a diffuse, pruritic rash with areas of necrosis, erosions, and blisters on the trunk, extremities, and face. It is important to note that the diagnosis of p-ANCA vasculitis with cutaneous involvement can be challenging, as the presentation can be like other conditions such as SJS, urticarial vasculitis, or even drug reactions. CONCLUSIONS: This case report emphasizes that physicians should consider p-ANCA vasculitis in the differential diagnosis of patients presenting with cutaneous lesions, especially when they have a history of autoimmune disease, resistant hypertension, lung and renal involvement. REFERENCE #1: 1. Marzano, Angelo Valerio, Maria Gabriella Raimondo, Emilio Berti, Pier Luigi Meroni, and Francesca Ingegnoli. "Cutaneous manifestations of ANCA-associated small vessels vasculitis. " Clinical reviews in allergy & immunology 53 (2017): 428-438. REFERENCE #2: McBride, Jeffrey D., and George W. Elgart. "Pyoderma gangrenosum–like facial ulcers in a woman associated with cocaine use and cANCA/anti-PR3+, pANCA/anti-MPO–serology." JAAD Case Reports 6, no. 9 (2020): 945-950. REFERENCE #3: Gupta, M., L. Duggal, N. Jain, and C. Bhavya. "Burkholderia sepsis mimicking flare of ANCA associated vasculitis: A rare presentation." (2020). DISCLOSURES: No relevant relationships by Noreena Aslam No relevant relationships by Karen Avgush No relevant relationships by Aamir Hayat