Two adult cases of a cutaneous lesion indistinguishable from typical infantile digital fibromatosis are added to the unique similar case so far reported in adulthood. The immunocytochemical localization of vimentin and muscle actin in the proliferating cells confirms their myofibroblastic nature and establishes closer relationships between the adult and the infantile variants of this entity. These two variants, however, appear to be clinically different, since all the adult cases were extradigital and did not recur after surgical excision. The term inclusion body fibromatosis underlines the histological hallmark of the lesion and should be used to identify this entity in place of recurrent infantile digital fibromatosis which does not seem any longer appropriate.
Intravascular lymphomatosis (IVL) is a rare, malignant B- or T-cell lymphoma with remarkable affinity for the endothelial cells of small vessels, particularly within the skin and central nervous system. It is a disease that mimics several neurological disorders, particularly those of cerebrovascular ischemic origin. The prognosis is generally poor, with a rapidly fatal outcome. As a result the diagnosis is often made at post-mortem. We report a rare case of a 73-year-old patient with IVL complicated by intracerebral haemorrhage. In literature two cases of systemic IVL complicated by intracerebral haemorrhage have been reported, but they presented initially with a disseminated intravascular coagulation (DIC). This is the first case of brain IVL complicated by intracerebral haemorrhage not associated to DIC. Increasing awareness of this disease as a differential diagnosis to a common clinical presentation may lead to more opportunities to evaluate new diagnostic and treatment approaches.
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