A Case of Leg Ulcer Resulting from Protein S Deficiency

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  • OTANI Ayako
    Department of Dermatology, Toho University Ohashi Medical Center
  • FUKUDA Hidetsugu
    Department of Dermatology, Toho University Ohashi Medical Center
  • NIIYAMA Shiro
    Department of Dermatology, Toho University Ohashi Medical Center
  • NAKAHASHI Sumie
    Department of Rheumatology, Toho University Ohashi Medical Center
  • NAGASHIMA Yoshinori
    Department of Rheumatology, Toho University Ohashi Medical Center Department of Cardiovascular Medicine, Toho University Ohashi Medical Center
  • AOYAMA Yukio
    Department of Anesthesiology, Toho University Ohashi Medical Center
  • MORISHITA Eriko
    Department of Pharmaceutical and Health Sciences, Kanazawa University Hospital
  • MUKAI Hideki
    Department of Dermatology, Toho University Ohashi Medical Center

Bibliographic Information

Other Title
  • プロテイン S 欠乏症による難治性下腿潰瘍の 1 例
  • 症例 プロテインS欠乏症による難治性下腿潰瘍の1例
  • ショウレイ プロテイン S ケツボウショウ ニ ヨル ナンチセイ カタイ カイヨウ ノ 1レイ

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Description

A 22-year-old man presented to our hospital with ulcers on his left leg, which had developed 9 months previously. He had been diagnosed with juvenile rheumatoid arthritis several years ago and had been prescribed 20 mg/day oral prednisolone and 5 mg/day warfarin. Physical examination revealed ulcers ranging from 1 × 1cm to 4 × 6 cm in size on his lower left thigh, surrounded by brown pigmentation. Organized necrosis and small, hard white nodules were visible at the base of the ulcers. Histopathological examination showed vein occlusion and calcium deposits in blood vessels and at the bottom of the ulcers. Ultrasonography of leg veins showed blood clots and valvular incompetence, and contrast-enhanced computed tomography (CT) of the legs revealed calcification of superficial veins. Blood tests revealed low protein S antigen and protein S activity (18％ and 10％ or lower, respectively) ; the patient was subsequently diagnosed with leg ulcers associated with protein S deficiency and treated with heparin, topical application of an anti-ulcer agent, straight leg raising, stockings and an artificial dermis skin graft, which showed an epithelization trend. Protein S deficiency should be considered as a possible cause of intractable leg ulcers in young people.

Journal

  • Nishi Nihon Hifuka

    Nishi Nihon Hifuka 77 (5), 461-464, 2015

    Western Division of Japanese Dermatological Association

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