a 62-year-old man, a car driving instructor complaining of thigh purpura NMC resident, General internal medicine

Transcription

1 a 62-year-old man, a car driving instructor complaining of thigh purpura MAKOTO INADA NMC resident, General internal medicine

2 Chief complaint purpura at right thigh History of Present Illness He had been well until 10 days before presentation, when he noticed purpura at his right thigh. At first he visited dermatology clinic, then he was referred to orthopedic hospital. There seemed no abnormal findings in skeletal systems, and then he was introduced to our hospital for the purpose of further medical examination, because he has anemia of hemoglobin level 11.7g/dL. During these 10 days purpura gradually spread to lower leg, and his right leg got swollen, accompanied with pain and restriction in range of knee motion. He doesn t remember any recent history of trauma nor infection. He denied standing or lying for a long time. He didn t have hematemesis nor tarry stool.

3 Past Medical History and Medication tetanus ( in childhood) gastroesophageal reflux disease allergic rhinitis taking mometasone 50μg 4 times nasal spray. constipation taking 1g magnesium oxide. no other medications, including OTC drugs, herbs, nor vitamin supplements. Allergy no known food and drug allergy. Familial Medical History unremarkable. no history of bleeding tendency. Social History Drink: never Smoke: current smoker. 1.5 pack for 40 years.

4 Physical examination Vital signs. BP 108/74 mmhg, PR 106/min RR 17/min SpO2 99%(ambient air) BT 37.0 (98.6 F) consciousness is alert. Height: cm Weight: 51.0kg BMI 17.7 He visited exam room on wheel chair because of pain and fatigue. HEENT: conjunctiva slightly pale, no jaundice. oral cavity was moist but loss of several teeth. dark stain on right mandibular gingiva. no lymphadenopathy, no goiter. HEART: S1 S2 S3(-)S4(-) no murmur. LUNG: Breath sound was clear to auscultation bilaterally, no rale. ABDMEN: flat and soft. no tenderness, no hepatosplenomegaly.

5 EXTREMITIES: On the right leg, there were ecchymoses on lateral side, and nonpalpable purpura consistent with follicles on the front side. On the other hand his left leg appeared normal. dark stain in gingiva ecchymoses at right leg purpura at right leg * leg photos were taken in day 5, oral cavity in day 8.

6 any Questions?

7 initial assessment day1 #1 purpura at right lower extremity s/o bleeding? #2 anemia d/t bleeding So, why is he bleeding at right leg? My differential is; V: blood vessels rupture, vasculitis, vascular problem I: skin infection like cellulitis, N: Haematologic malignancy. D: Liver cirrhosis, C: hemophilia, platelet abnormal function, factor ⅩⅢ deficiency A: Henoch-Schonlein purpura, vasculitis, SLE, acquired haemophilia T: Trauma,

8 blood test WBC 5900 /μl seg 61.8 % Lym 26.9 % mono 5.6 % eos 4.7 % baso 1.0 % Hb 9.3 g/dl MCV 98 fl MCHC 33.3 % Plt 27.0 *10^4/μL bleeding time 1 min reticulocyte 14 Fe 70 μg/dl TIBC 239 μg/dl Ferritin 67.1 ng/ml Na K BUN CRE Alb AST ALT ALP T.bil LD CPK PT APTT D dimer CRP 137 meq/l 4.7 meq/l 11 mg/dl 0.68 mg/dl 3.6 g/dl 16 IU/L 7 IU/L 176 IU/L 1.4 mg/dl 242 IU/L 70 IU/L 14.6 sec 28.6 sec 1.6 μg/dl 1.52 mg/dl

9 Chest X-ray bullas in both upper lung field. no apparent mass, no consolidation.

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11 secondary assessment day1 #1 purpura #2 anemia Hgb in 6 days laboratory exam showed normal number of platelets, bleeding time, APTT and slightly elongated PT. He has no familial history and past medical history of bleeding tendency so my differential is; vasculitis, vascular problems, collagen problem, factor ⅩⅢ deficiency, abnormalities of platelet function such as von Willebrand disease. On the other hand, reticulocyte index is 0.58, and MCV is relatively high within normal limit. Vitamin B12 and folic acid was added.

12 additional test (reference) ANA <40 (<40) PR3-ANCA <1.0 U/ml (<3.5) MPO-ANCA <1.0 U/ml (<3.5) C3 121 mg/dl (73-138) C4 29 mg/dl (11-31) ⅩⅢ coagulation 82 % ( ) IgG 1631 mg/dl ( ) IgA 364 mg/dl (93-393) IgM 46 mg/dl (33-183) VB pg/ml (18-914) folic acid 1.2 ng/ml (>4.0) Urinalysis gravity ph 6.0 glucose <1.0 protein <1.0 occult blood 2+ bilirubin (-) Urobilinogen (±) WBC reaction (-) nitrite (-) RBC sediment /HPF WBC sediment 1-4 /HPF epithelium 1 /HPF hyaline cast 1 /HPF

13 CAT scan: Subdermal edematous change but no signs of arterial bleeding. There are bullas in both lungs, however there is no apparent malignancy in chest and abdominal region.

14 tertiary assessment day5 #1 purpura d/t vascular problem #2 anemia Hgb purpura was still worsening, and anemia progressed. Laboratory exam showed no apparent signs of vasculitis, autoimmune diseases. Does he have vasculitis? Any collagen or vascular problems? By the way folic acid is lower than usual. And why? Does he have any nutritional problem?

15 What kind of meals do you often take? I usually have canned fish with rice, sometimes instant noodles with egg. I never eat vegetables because I don t like. I see. So, I need additional blood test and you need vegetables. Ok, I ll try it.

16 additional test Vitamin C <0.2 μg/ml (reference μg/ml) clinical diagnosis SCURVY

17 clinical course Treatment: Vitamin C 600mg supplement as well as folic acid were prescribed. Education: appointment to visit nutritionist. She asked kinds of vegetables he could eat and encouraged him to take them. He began to have spinach, cabbages, carrots and onions. Next week(day14), His Hgb level improve from 8.1 to 9.0 g/dl, and his symptoms improved. His leg pain was alleviated and he could move easier than before.

18 day 5 day 8 day 36 day 15 Hgb (g/dl) Vitamin C 3.5 folic acid days

19 SCURVY uptodate, overview of water-soluble vitamins

20 SCURVY and Vitamin C Vitamin C(ascorbic acid) deficiency Scurvy Symptoms: petechiae, perifollicular hemorrhage, bruising, gingivitis, arthralgia, impaired wound healing, appearing within a few months. Ascorbic acid is involved in such biologic processes as collagen synthesis. History: In the age of Discovery, plenty numbers of sailors died of scurvy. In mid 18th century, British Naval surgeon James Lind showed that the scurvy patients who took orange recovered soon. In 20th century, Hungarian physiologist Szent-Györgyi Albert identified anti-scurvy factor and named it Vitamin C.

21 Lind s clinical study (1747) James Lind, Naval surgeon ( ) Scurvy Navy orange vinegar sulfuric acid with EtOH sea water apple liqueur herbs

22 skin and oral abnormality in scurvy perifollicular hemorrhage gingival abnormality uptodate, overview of water-soluble vitamins

23 risk factor and diagnosis Risk factor severely malnourished individuals, such as; drug or alcohol abuse poverty diets devoid of fruits and vegetables. children with autism iron overload Diagnosis High performance liquid chromatography.(biochemistry) Symptoms of scurvy generally occur when the plasma concentration of ascorbic acid is less than 0.2mg/dl(2.0μg/ml).

24 treatment and daily requirement Adult patients are treated with 300mg-1000mg daily for one month. in Japan 100mg daily is recommended for regular dietary intake. Dietary intake: a lemon has about 20mg Vitamin C. Vitamin C rich vegetables or fruits are like; peppers, bitter gourd(a.k.a. Goya), kiwi fruits, acerola, seaweed.

25 Vitamin C content of snacks he ate canned Sanma fish 0mg/100g rice 0mg/100g instant noodle 0mg/100g egg 0mg/100g * Japanese food standard ingredient table

26 Take Home Message ecchymoses with normal coagulation, platelet count and function suggests vascular problem. nutrition problems are not past diseases. Don't be picky!

27 Dr. Tierney s pearl. In differential of poor healing fracture in old/old, remember scurvy