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cutaneovisceral angiomatosis

Sunday 28 January 2007

Definition: Cutaneovisceral angiomatosis associates multiple vascular lesions of the skin and gastrointestinal tract with sustained, minor thrombocytopenia.

Synopsis

- multiple vascular lesions of the skin and gastrointestinal tract
- sustained, minor thrombocytopenia
- involvement of the lung, bone, liver, spleen, and muscle
- thin-walled, blood-filled vascular channels
- variable endothelial hyperplasia
- endothelial nuclei elongated, round, crescentic, or hobnailed
- cytoplasmic and extracellular periodic acid-Schiff positive deposits often present in the zones of endothelial hyperplasia
- gastrointestinal hemorrhage
- hemoptysis

See also

- congenital proliferative disorder of blood vessels
- cutaneovisceral angiomatosis with thrombocytopenia

References

- Prasad V, Fishman SJ, Mulliken JB, Fox VL, Liang MG, Klement G, Kieran MW, Burrows PE, Waltz DA, Powell J, Dubois J, Levy ML, Perez-Atayde AR, Kozakewich HP. Cutaneovisceral angiomatosis with thrombocytopenia. Pediatr Dev Pathol. 2005 Jul-Aug;8(4):407-19. PMID: 16034654