Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Net letter
2013:79:5;725-725
doi: 10.4103/0378-6323.116736
PMID: 23974600

Edema and ulcers on the legs and dilated abdominal veins caused by thromboembolism of inferior vena cava in a patient with protein C deficiency

Hideo Kawano, Chika Ohata, Fumitake Ono, Norito Ishii, Takahiro Hamada, Takekuni Nakama, Minao Furumura, Daisuke Tsuruta, Takashi Hashimoto
 Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan

Correspondence Address:
Takashi Hashimoto
Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, 67 Asahimachi, Kurume, Fukuoka 830-0011
Japan
How to cite this article:
Kawano H, Ohata C, Ono F, Ishii N, Hamada T, Nakama T, Furumura M, Tsuruta D, Hashimoto T. Edema and ulcers on the legs and dilated abdominal veins caused by thromboembolism of inferior vena cava in a patient with protein C deficiency. Indian J Dermatol Venereol Leprol 2013;79:725
Copyright: (C)2013 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

The major clinical symptom of protein C deficiency is venous thromboembolism, which mostly occurs in deep veins of the lower extremities, but can also cause thrombosis in inferior vena cava. [1] We report a case of inferior vena cava thrombosis caused by Protein C deficiency, which subsequently caused leg ulcers without deep vein thrombosis (DVT) of the lower extremities.

A 33-year-old Japanese male cook noticed pain in the legs and the lower back and visited an orthopedist. Computed tomography (CT) revealed thrombosis of inferior vena cava, just below bifurcation area of renal vein, left iliac vein, left femoral vein, and left greater saphenous vein. Subsequently, the patient was referred to cardiovascular department in our hospital, and was diagnosed as having hereditary protein C deficiency, which was considered as a cause of thrombosis. Patient had a family history of cerebral infarction in his father. Anticoagulant therapy was started; however, the patient discontinued all treatments 6 months later. The patient had no treatment for approximately 5 years, and then noticed edema on the legs, followed by slowly developing ulcers on the right leg. The patient visited us again.

Physical examination revealed symmetrical scaly erythema on the both legs with extensive edema [Figure - 1]. Two skin ulcers 20 mm in size were found within the erythema on the inner surface of the right leg [Figure - 1]. In addition, extensively dilated epigastric veins were shown on the abdomen [Figure - 2]. Neither livedo nor varix was recognized on the both legs. Laboratory tests revealed decreased activated protein C (58%, normal range 64-146) and protein C antigen levels (45%, normal range 70-150). The results of other coagulation tests including D-dimer and activated Protein S were within normal limits. Antinuclear antibody, anti-cardiolipin antibody, lupus anticoagulant, myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA), and proteinase 3 -ANCA were not detected. Chest, abdomen, and leg contrast enhanced CT revealed obstruction of inferior vena cava below the level of diaphragm and bilateral common iliac veins with several collateral veins, including mesenteric veins, subcutaneous veins of the abdomen and paravertebral veins [Figure - 3]. There was neither DVT in the legs nor pulmonary embolism. Skin biopsy from the edge of the ulcer revealed dilation and proliferation of the capillaries with thick walled vessels in the superficial dermis. There was no thrombus formation in the specimen. We diagnosed leg ulcers due to venous stasis in association with inferior vena cava thrombosis caused by congenital heterozygous protein C deficiency. Re-started anticoagulation therapy with warfarin sodium cleared the ulcers dramatically along with the improvement of extensive edema and scaly erythema on both legs in 3 weeks.

Figure 1: Clinical appearance. Two skin ulcers 2 cm in size with erythema on the right inner leg
Figure 2: Multiple dilated subcutaneous veins in the abdomen
Figure 3: Transverse contrast-enhanced computed tomography revealed obstruction of inferior vena cava (arrow) and dilated collateral veins in superficial abdominal wall (arrow head)

In our case, protein C deficiency first caused obstruction of inferior vena cava, and subsequently, edema of the legs with leg ulcers and dilated abdominal veins developed. The most common physical signs of chronic inferior vena cava obstructions are bilateral edema of the legs and dilated superficial abdominal wall collateral veins. [2] However, a review of 24 patients with inferior vena cava obstruction revealed bilateral leg edema in only 10 (42%) patients, and dilated abdominal veins in 12 (50%) patients. [3] Out of 24, 3 patients developed bilateral leg ulcers without DVT on the leg. Among these 3 patients, only one patient was accompanied with bilateral leg edema and dilated abdominal veins. Thus, concurrence of edema and ulcers on the legs and dilated abdominal veins due to obstruction of inferior vena cava without DVT on the leg, which was exactly the same condition in our case, seems to occur rarely.

Our patient was a cook, and spent a long time in a standing position. Therefore, the patient′s life-style is probably responsible for the disease development; although, external injury could also be a trigger for the skin ulcers.

For the treatment of heterozygous protein C deficiency, oral anticoagulant with a coumarin-derivative or heparin remains standard therapy. [4] In our patient, both edema and ulcers on the legs were cured only 3 weeks after initiation of anticoagulant therapy. Furthermore, thrombosis found in left femoral vein and left greater saphenous vein in the first CT disappeared in the second CT, 6 months after anticoagulant therapy. These results signify that our patient responded well to anticoagulant therapy. However, the delay of initiation of the treatment might have led to Budd-Chiari syndrome, the most severe complication of protein C deficiency, because thrombosis extended to the level of the diagram in the second CT. [5]

Laboratory tests and physical examination excluded leg ulcers caused by venous valve dysfunction, artheriosclerosis, arterial embolism, arteritis, and phlebitis. Although some patients with protein C deficiency develop warfarin induced necrosis, our patient did not.

Acknowledgments

We gratefully appreciate the secretarial work of Ms. Hanako Nakagawa and Ms. Sachika Notomi. We thank the patient for his participation.

References
1.
Kobayashi T, Ito A, Okada Y, Kojima N, Fujita A, Teruya M. Protein C deficiency as a cause of simultaneous acute thrombosis of the superior mesenteric vein and inferior vena cava with jejunal infarction. Surgery 2005;137:482-3.
[Google Scholar]
2.
Missal ME, Robinson JA, Tatum RW. Inferior vena cava obstruction: Clinical manifestations, diagnostic methods, and related problems. Ann Intern Med 1965;62:133-61.
[Google Scholar]
3.
Jackson BT, Thomas ML. Post-thrombotic inferior vena caval obstruction. A review of 24 patients. Br Med J 1970;1:18-22.
[Google Scholar]
4.
Pescatore SL. Clinical management of protein C deficiency. Expert Opin Pharmacother 2001;2:431-9.
[Google Scholar]
5.
Hiroe S, Itoh H, Matsumoto H, Takahasi S, Sato Y, Yamada S, et al. Case of Budd-Chiari syndrome 3 months after vaginal delivery. J Obstet Gynaecol Res 2008;34:605-8.
[Google Scholar]

Fulltext Views
248

PDF downloads
77
Show Sections