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Indian Journal of Dermatology, Venereology and Leprology
Medknow Publications on behalf of The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL)
ISSN: 0378-6323
EISSN: 0973-3922
Vol. 73, No. 6, 2007, pp. 412-414
Bioline Code: dv07155
Full paper language: English
Document type: Case Report
Document available free of charge

Indian Journal of Dermatology, Venereology and Leprology, Vol. 73, No. 6, 2007, pp. 412-414

 en Tuberous sclerosis with portal vein thrombosis, protein C and S deficiency
Vasudevan, Biju; Sawhney, M.P.S; Radhakrishnan, S & Shilpa, G

Abstract

A 29-year-old lady with a bad obstetric history and portal vein thrombosis, presented to the Skin OPD for facial lesions. On examination, angiofibromas on face, shagreen patch and periungual fibromas were observed. She also had dental pits and a retinal hamartoma. Investigations revealed hamartomas in the brain and kidney. Hematological work-up showed protein C and S deficiency with Factor V Leiden positivity. Except for the cutaneous symptoms, the patient did not have any clinical manifestations in other organs affected by tuberous sclerosis. A similar association of tuberous sclerosis with protein C deficiency has been reported in only one case in literature.

Keywords
Tuberous sclerosis, Protein C deficiency, Protein S deficiency

 
© Copyright 2007 Indian Journal of Dermatology, Venereology and Leprology.
Alternative site location: http://www.ijdvl.com

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