AB0767 SEVERE BREAST ULCERATION IN BEHÇET’S DISEASE TREATED WITH CYCLOPHOSPHAMIDE: A CASE REPORT

Abstract

Background:Behcet’s disease is a systemic autoimmune vasculitis that typically involves mucocutaneous surfaces, the eyes and the skin with varying manifestations. Skin ulceration often secondary to pathergy is a common feature.Objectives:To raise awareness of breast ulceration as a clinical manifestation in Behcet’s disease.Methods:We report a case of complex Bechet’s disease associated with severe refractory breast ulceration successfully treated with cyclophosphamide.Results:A 27 year old female patient presented to the rheumatology department with a non-healing lesion on her face secondary to a cat scratch. She reported previous episodes of ulceration usually as a result of minor trauma such as IV cannulation, as well as a two year history of severe oral and genital ulceration. She had also been diagnosed with ‘colitis’ requiring iliocaecal resection two years prior to presentation. Previous treatments for her bowel included azathioprine, infliximab, adalimumab and vedolizumab. Over the next three years she developed recurrent eye involvement in the form of scleritis with ongoing oral and genital ulceration. Further treatment under our care included ustekinumab, entanercept, benepali, certolizumab and tacrolimus with background prednisolone. She then developed a small area of broken skin on her left breast which continued to extend and ulcerate despite treatment with IV steroids. Tissue biopsy showed deep focal vasculitis with intravascular thrombi. Anticoagulation was commenced and she was treated with tocilizumab IV for 3 months with no clinical improvement. Involvement extended to include 80 percent of the breast surface and the nipple self-amputated. Due to the severity and extent of the ulceration which was extremely painful and distressing, she was given 3 months of IV cyclophosphamide at a dose of 15mg/kg every 2-3 weeks. The ulceration showed rapid clinical improvement (see Figure 1).Figure 1.Breast ulceration before and after 3 months of cyclophosphamide treatmentConclusion:Involvement of the breast in systemic vasculitides (such as GPA or PAN) has been reported, although usually manifesting as a palpable mass with diagnosis on biopsy 1. Similar presentations in Behcet’s disease have been recognised 2, although much less commonly. Our case demonstrates that severe breast ulceration can be a clinical manifestation of this condition and can be refractory to usual therapies.References:[1]Ren J, Liu J, Su J, Zhang J, Zhao J. Systemic vasculitis involving the breast: a case report and literature review. Rheumatol Int. 2019 Aug;39(8):1447-1455[2]Soleto MJ, Marcos L. Behçet’s disease involving the breast. Eur Radiol. 2002 Dec;12 Suppl 3:S98-S100. doi: 10.1007/s00330-002-1420-4.Disclosure of Interests:None declared