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AJNR - American Journal of Neuroradiology

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Nervous System Involvement in Degos Disease

Carmelo Amato^a, Raffaele Ferri^b, Maurizio Elia^b, Filomena Cosentino^b, Carmelo Schepis^c, Maddalena Siragusa^c and Massimo Moschini^d

^a Department of Neuroradiology, Oasi Institute for Research on Mental Retardation and Brain Aging, Troina, Italy
^b Department of Neurology, Oasi Institute for Research on Mental Retardation and Brain Aging, Troina, Italy
^c Department of Dermatology, Oasi Institute for Research on Mental Retardation and Brain Aging, Troina, Italy
^d Department of Neuroradiology, Catholic University, Rome, Italy

View larger version (72K): [in a new window]   FIG 1. Typical skin lesions of Degos disease. Papules on patient leg, at different stages, with whitish, atrophic center and erythematous rim.

View larger version (73K): [in a new window]   FIG 2. Skin histologic specimen showing atrophic epidermis with no crests (A, arrow) and low cellularity and focal necrosis in underlying dermis (Hematoxylin-eosin). High magnification of deep dermis (B) revealing arteriolar hyalinosis with endothelial hyperplasia (arrowheads) and perivascular lymphomonocitic infiltrate (arrows).

View larger version (79K): [in a new window]   FIG 3. Contrast-enhanced T1 axial images show multiple nodular lesions located in the peripheral gray, subarachnoid spaces and leptomeninges, with diffuse dural enhancement. Note the ependymal enhancement (A) of the right atrium and a small infarct in the left caudate head.

View larger version (175K): [in a new window]   FIG 4. Contrast-enhanced FLAIR coronal image shows cerebral and cerebellar lesions, right ependymal alteration, and dural thickening; ischemic changes can be noted in the left parietal white matter. On the right, subtle hypointense CSF collection is recognizable among thickened meninges.

View larger version (77K): [in a new window]   FIG 5. T2 (A) and T1 (B, contrast-enhanced) axial images at supraventricular level show right thickened meninges, subdural CSF collection (right side more evident than left) and multiple superficial lesions.

View larger version (92K): [in a new window]   FIG 6. T2 sagittal image shows diffuse spinal cord thinning and midthoracic focal hyperintensity.

View larger version (128K): [in a new window]   FIG 7. T1 (contrast-enhanced) sagittal images show subtle cord enhancement in the dorsal cervical region and at midthoracic level (arrows).