PMC7154106_01
(A) Macroscopic view of medium and dark-blue venous nodules affecting the patient's forehead. (B) Skin-colored compressible protuberances and a small, dark-blue venous nodule affecting the right forearm. (C) A sagittal view of a cranio-cervical computed tomography scan shows heavy, hyperdense, calcified lesions centered by a hypodensity. The diffuse white matter hypodensity represents severe edema with mass effect. (D) Hematoxylin and eosin staining of the lesions show light areas of dilated venous vessels and congestive, thin-walled veins internally coated by a layer of flat endothelial cells. The vascular channels are arranged in the superficial, middle and deep reticular dermis.. Written informed consent was obtained from the participants for the publication of this case report.
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F1
Nodular neoformations were rubbery, adherent, and seemed to be vascular in nature; skin biopsies were taken (Figures 1A,B). A brain computed tomography (CT) scan revealed a large, right frontal hematoma in acute and subacute phases with perilesional edema and no deviation of the mid-line structures, multiple calcified intraparenchymal lesions, as well as a punctiform hemorrhage within the medulla oblongata (Figure 1C). A separation into inner or outer layers by an aberrant elastic lamina was also observed (Figure 1D).
(A) Sagittal section of the encephalon shows a cortico-subcortical intraparenchymal hemorrhage that destroyed the basal ganglia. A nodular lesion in the temporal cortex corresponds to an AVM (arrow). (B) Malformed and irregular intracortical vessels. Masson's trichrome stain x. (C) Axial T2-weighted MR (magnetic resonance) and gradient echo (GRE) images: bilateral supratentorial hypointense lesions, harboring a central hyperintensity, 1.2 to 1.6 cm. A heterogeneous tumoral lesion can be seen in the right frontal lobe (5.38 x 5.4 cm), with mass effect and medial displacement of both basal ganglia and ipsilateral ventricle. (D) A coronal view of a T1-weighted image with contrast shows hyperintense lesions, primarily located in the right frontal lobe, and a similar lesion in the temporal pole corresponding to AVM (arrows).
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F2
This pattern was consistent with CCM:like lesions (Figures 2C,D). Additionally, there was intraparenchymal hemorrhage at the basal ganglia (Figures 2A,B).
PMC7154106_02
(A). Macroscopic view of skin-colored compressible protuberances in the cervical neck region simulating a lipoma (Blue arrow). (B) Macroscopic view of the left side of the chest showing small, dark-blue venous nodules (Blue arrow). (C,D) Axial gradient echo images, multiple lesions shown by red arrows.
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F3
Brain MRI T2-weighted GRE revealed multiple hypointense lesions, suggestive of CCM-like, with no associated acute complications (Figure 3).