Indian Journal of Dermatology, Venereology and Leprology
Medknow Publications on behalf of The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL)
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
© Copyright 2007 Indian Journal of Dermatology, Venereology and Leprology.
Tuberous sclerosis with portal vein thrombosis, protein C and S deficiency|
Vasudevan, Biju; Sawhney, M.P.S; Radhakrishnan, S & Shilpa, G
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.
Tuberous sclerosis, Protein C deficiency, Protein S deficiency
Alternative site location: http://www.ijdvl.com