PO:25:081 | Atypical onset of anti-SAE-1 positive dermatomyositis: paraneoplastic flame hemorrhages in urothelial carcinoma
Abstract
Background. Flame hemorrhages are a manifestation of infectious endocarditis, as well as autoimmune or neoplastic diseases. Anti-SAE-1 antibodies (small ubiquitin-like modifier-activating enzyme, against enzymes involved in gene transcription mechanisms and apoptosis control) are specific for a form of dermatomyositis with mild skin and muscle involvement that can be associated with neoplasms.
Materials and Methods. We describe the case of a 50-year-old patient who developed flame hemorrhages and acrocyanosis with positivity for anti-SAE-1 antibodies.
Results. Following a urinary tract infection, treated with levofloxacin, she developed Raynaud's phenomenon, flame hemorrhages, and acrocyanosis (Figure 1). Increased PCR and ESR (1.42 mg/dL and 45 mm/h), blood count and biochemistry within normal limits. An upper limb Doppler ultrasound and a chest CT scan ruled out vascular pathologies of the upper extremities, and a transesophageal echocardiogram ruled out infectious endocarditis. Video capillaroscopy showed the absence of a frank scleroderma pattern but the presence of multiple flame hemorrhages and thrombosed capillaries (Figure 2). Regarding autoimmunity: ANA positive, fine dense speckled cytoplasmic (AC-19), anti-ENA, ANCA, APL, and LAC absent. Anti-SAE-1 tested positive on the rare myositis antibody panel. Acrocyanosis was treated with an iloprost infusion, with partial benefit. During the first-level paraneoplastic screening, a third-degree hydronephrosis of the left…
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