Proteus syndrome is an extremely rare disorder that manifests as an asymmetric, disproportionate overgrowth of any connective tissue, such as bone, fat or epidermal nevi, in a mosaic or patchy pattern. It has an estimated prevalence of less than 1/1,000,000 live births. The diagnosis can be difficult because the phenotypes of the patients are variable. Many individuals develop cutaneous capillary malformation and prominent varicosities (large and complex vascular malformations). Thus, Proteus syndrome patients are at risk of developing deep vein thrombosis and pulmonary embolism. The authors present the case of a patient with Proteus syndrome who was admitted because of pulmonary thromboembolism and presented hypertrophy of the left arm and left hemithorax.
Polyarteritis nodosa is a systemic necrotizing vasculitis that typically affects medium-sized muscular arteries, with occasional involvement of small muscular arteries. Most cases of polyarteritis nodosa are idiopathic but multiple infectious agents have been associated with this disease. We present a clinical case of a 72-year-old male with fever, diarrhoea and haemodynamic instability, diagnosed with a bacterial infection caused by Salmonella Typhi. One week after clinical resolution of the infection, the patient developed purpuric lesions with ulcers, pustules and necrotic areas accompanied by testicular pain and weight loss of 5 kg over the previous 15 days. A skin biopsy was performed and it revealed typical histologic signs of polyarteritis nodosa. The aetiologic association between bacteria of the genus Salmonella and polyarteritis nodosa has been previously described in the scientific literature but seldom meeting classification criteria and with histologic confirmation.
procedures followed were in accordance with the regulations of the relevant clinical research ethics committee and with those of the Code of Ethics of the World Medical Association (Declaration of Helsinki). Provenance and Peer Review: Not commissioned; externally peer reviewed. © Autor (es) (ou seu (s) empregador (es)) e Revista SPMI 2021. Reutilização permitida de acordo com CC BY-NC. Nenhuma reutilização comercial.
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