This document describes a case of a 43-year-old male farmer with end-stage renal disease and hypertension who presented with left foot pain and a left big toe ulcer. Examination found erythema of both feet. Tests showed elevated parathyroid hormone, calcium, and phosphate levels consistent with calciphylaxis. The patient was treated with calcium-lowering drugs and prepared for parathyroidectomy to reduce further complications from calciphylaxis, a condition of vascular calcification and skin necrosis seen in long-term kidney disease.

2. By
Mahmoud Samir Foda
Ass. Lecturer of Internal Medicine and Nephrology
Faculty of Medicine
Tanta University

4. Male patient, 43 years old, HTN and ESRD maintained
on regular HD from AVF 6 years ago in HD unit at Tanta
University Hospital.
He is farmer from rural area, married with no special
habits.
Complaint:
 Feet pain mainly in lt foot since 9 months ago.
 left big toe ulcer 1 month ago.

5. History of present illness:
 The complaint started 9 months ago with gradual onset
and progressive course of feet pain mainly on lt foot pain
burning in character ↑ by touch and dependent position, ↓
by elevation with little improvement by analgesics.
 8 months later patient developed left big toe ulcer begin
to appear with erythema of the other toes and erythema of
the Right foot. The patient presented also by Pain in Rt
thigh, weakness and inability to go upstairs.

6. HD session prescription:
• Session 2.5 hour,3 times /week , pump 270 ml/minute ,
low flux polysulphone 1.2 m2 hemodialyzer using
commercially available dialysate with average Kt/v 0.9.

7. Past history :
 2 years after starting the dialysis, kidney transplantation was
done (1 year and 3 months without dialysis), but unfortunately
chronic graft rejection occurred and the patient had
hemodialysis again.
 HTN since 7 years.
 history of Cellecpt and Corticosteroids intake during the
period of transplantation .
Family history :
Irrelevant

8. Drug history
 Calcium acetate 2gm/day.
 Alphacalcidol 1 micogm/day.
 Multivitamins.
 Amlodipine 5 mg/day.
 IV iron sucrose.

10.  The patient was conscious, pale with uremic facies,
average body built and no special attitude in bed.
 Vital signs: blood pressure: 110/70 mmHg.
pulse: 90 b/m, regular, average volume &
dorsalis pedis pulsation felt bilaterally.
Temp: 37 C.
R.R: 14 c/m.
 Abdominal examination: ascites.

11.  Extremities: - No lower limb edema.
- left big toe ulcer ( oval in shape ,elevated
margins, with dirty floor) erythema of both
feet.
 Chest: Unremarkable
 Heart: Unremarkable

12. pictures of the patient

15.  Liver function tests:
Bilirubin= 1 mg/dl
AST= 30 IU/L ALT= 25 IU/L.
S. albumin= 3 g/dl.
 CBC:
• Hb= 10 g/dl (microcytic hypochromic).
• platelets= 150,000/mm³.
• WBCs= 10,000/mm³ (Neut. 88%).
• ESR: 1st h= 60 mm 2nd h= 95 mm.
 Prothrombin activity: 95%. APTT: 37 sec.

16.  S. creatinin: 4.4 mg/dl.
 B. urea: 66 mg/dl (before) 35 mg/dl (after).
 URR: 46.9 %
 Uric acid: 6 mg/dl.
 FBG: 100 mg/dl. PPBG: 130 mg/dl.
 Viral markers:
• HBs Ag: Negative.
• HCVAb: positive.
• HIV Ab : Negative.
 S.Ferritin= 25.9 ng/ml. TSAT% :10%.

17.  Electrolytes & autoimmune markers:
• Na= 132 mmol/L
• K= 4.5 mmol/L
• Po4= 6.0 mg/dl
• i Ca= 0.9 mmol/L
 PTH= 3100 pg/ml
 ANCA (p&c)= -ve
 ANA= -ve
 Anti Ds DNA= -ve

18.  Pelvi-Abd US: Atrophic renal graft and moderate ascites.
 Chest x ray: - NAD

21. DD
 systemic vasculitis.
 peripheral vascular disease.
 Atheroemboli.
 warfarin-induced skin necrosis.
 Cryoglobulinemia.
 Calcific Uremic Arteriolopathy(Calciphylaxis).
 Hypercoagulability as protein c def.

23. • Serum Cryoglobline :-ve.
• Rheumatoid factor:-ve .
• Alkaline phosphatase:233u/l (N upto 187)
• protein C level, protein S level, anticardiolipin level,
lupus anticoagulant level: normal.

25. Lateral x- ray on the abdomen

29. • Echocardiography :
average L V internal dimensions with
good systolic function EF=74% with
diastolic dysfunction, Dilated left
atrium.

30. X –ray on the hands and the feet

35. Duplex on the arterial and venous
system of both lower limbs
• Diffuse arterial atherosclerotic
changes with chronic occlusion of the
tibial arteries.

37. • The patient received cinacalcet 30mg bid for 2
weeks then once daily and sevelamer 2.4 gm/d.
His investigations after 2 weeks and 1
month
• ionized Ca 1.0 →0.99 mmol/L
• PO4 5.3 → 3.4mg/dL
• PTH 2513 →1489 pg/mL.

38. • After stopping of cinacalcet there is rising in the PTH level
(PTH=2980 pg/mL) and now the patient is
prepared for parathyrodectomy.

40. DEFINATION
• Calciphylaxis is a poorly understood
and highly morbid syndrome of
vascular calcification and skin necrosis.

41. DIAGNOSIS
 History:
• A long-standing history of CKD and renal
replacement therapy.
• Very rarely, it may occur in an individual without a
history of chronic renal failure.
• Many persons who develop calciphylaxis have
undergone renal allograft transplantation.

42. • There are triggers to calciphylaxis :
 Long-term obesity.
• Infusion of medications such as iron dextran.
• Remote and/or recent use of immunosuppressive agents,
especially corticosteroids.
• Diabetes mellitus and insulin injections.
• Use of vitamin D and calcium-based phosphate binders.
• Elevated aluminum levels.
• Concomitant vascular disease.

43. • Concurrent use of warfarin anticoagulation:
Current data suggest that warfarin therapy may
lower protein C concentrations, leading to a
procoagulant condition in the calcified vessel.
• Warfarin may also inhibit carboxylation of matrix
Gla protein, an important inhibitor of calcification,
thus promoting calcification.

44. EXAMINATION
• Early lesions of calciphylaxis manifest as
nonspecific violaceous mottling; as livedo
reticularis; or as erythematosus papules, plaques,
or nodules.
• More developed lesions have a stellate purpuric
configuration with central cutaneous necrosis .

45.  Lesions may be singular or numerous, and they
generally occur on the lower extremities however,
lesions also may develop on the hands.
 Intense pain is a constant finding.
 An intact peripheral pulse helps to distinguish acral
calciphylaxis from atherosclerotic peripheral vascular
disease.

46. TREATMENT
• Reduce serum phosphate level.
• Limit excessive calcium loading.
• Reduce vitamin D analog dose in patients with
suppressed PTH levels.
• Calcimimetics ?? in patients with elevated PTH
levels.
• Parathyroidectomy.