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• W2414896687 abstract "Case ReportCalciphylaxis in a 33-year-old man with end-stage renal disease Khalid M. Al Ghamdi Khalid M. Al Ghamdi Search for more papers by this author Published Online:6 Oct 2005https://doi.org/10.5144/0256-4947.2005.415SectionsPDF ToolsAdd to favoritesDownload citationTrack citations ShareShare onFacebookTwitterLinked InRedditEmail AboutIntroductionCalciphylaxis is an uncommon and poorly understood cutaneous phenomenon associated with chronic renal failure (CRF). The cardinal features are cutaneous purpura and necrosis as well as histopathologic findings of calcifications of cutaneous vessel walls. There is significant morbidity from this disease, which is mainly caused by overwhelming sepsis.1Case ReportA 33-year-old man known to have end-stage renal disease (ESRD) of unknown cause who had kidney transplantation twice was referred to the dermatology clinic in November 2002 with a one-week history of rash over both thighs associated with a severe burning sensation.The patient was diagnosed with ESRD requiring haemodialysis in 1988. He underwent a first renal transplantation (living, unrelated) in India in 1991 and had a rejection after 3 years, which required a return to haemodialysis. Four months prior to presentation he had a second renal transplantation in Iran (living, unrelated), which also rejected after 6 weeks. As a consequence he was put on hemodialysis again. This patient had severe hyperparathyroidism as well. Current medications were oral prednisolone 17.5 mg once daily, oral tacrolimus 2 mg twice daily, oral ferrous sulphate 325 mg thrice daily, gancyclovir 500 mg orally thrice weekly, amlodipine 5 mg orally once daily, omeprazole 20 mg orally once daily, prazocin hydrochloride 2 g orally thrice daily and aluminum hydroxide 2 tablets thrice daily.Physical examination revealed multiple purpuric macules and papules over the medial aspect of both upper thighs down to the knees. The legs and buttocks were not involved. Systemic examination was unremarkable. Blood work showed a low haemoglobin and haematocrit (Table 1). Mean corpuscular volume, mean corpuscular haemoglobin, white blood cell count, platelets count, bilirubin level, prothrombin time, and partial thromboplastin time were normal. Parathyroid hormone was very high. Serum urea and serum creatinine were elevated. Serum sodium, chloride, protein C, protein S and fasting glucose were within normal limits. Total calcium was normal, but due to low albumin, the corrected calcium was high. Serum phosphate was elevated, and accordingly, the calcium phosphorus product was high. Urinalysis is shown in Table 1. HIV serology was negative. X-ray of lower limbs showed a significant calcification of blood vessels.Table 1 Laboratory parameters.ParameterValue (normal range)Hematology Haemoglobin (g/L)78 (132–172) Haematocrit (%)0.248 (0.38–0.52) Parathyroid hormone (ng/L)1423 (16–65) Serum urea (mmol/L)19 (4.2–7.2) Serum creatinine (μmol/L)500 (65–129) Total serum calcium (mmol/L)2.45 (2.1–2.55) Serum albumin (g/L)24 (32–48) Corrected serum calcium (mmol/L)2.85 Serum phosphate (mmol/L)2.05 (0.7–1.45) Calcium phosphorus product5.84 (≤4.4)Urinalysis pHNormal Blood3+ Bilirubin and ketoneNegative Leukocytes2+ White blood cells (/hpf)>50 Red blood cells (/hpf)>50 Epithelial cells (/hpf)>50 Glucose1+Initial skin histopathological examination showed intradermal hemorrhage but no evidence of vasculitis. Two weeks later, the patient showed up in clinic with more progressive lesions, which were very painful. There were multiple large subcutaneous indurated plaques covered with multiple small hemorrhagic vesicles and black eschar over both thighs (Figure 1). A second skin biopsy revealed that the epidermis, dermis and subcutaneous tissue were necrotic and there was a significant amount of microthrombi. Several subcutaneous vessels contained calcium deposits in their walls (Figure 2). This picture is consistent with calciphylaxis. Bacterial, fungal and mycobacterial cultures were all negative.Figure 1 Bilateral well-defined black necrotic eshcar over the medial aspect of both lower limbs (top); Close-up view showing the black necrotic eschar with two small tense vesicles (bottom).Download FigureFigure 2 Intravascular calcium deposition with microthrombi in adjacent smaller blood vessels (hematoxilyn and eosin stain × 40).Download FigureThe patient was admitted to hospital and had a parathyroidectomy. Histopathological examination revealed hyperplasia of all parathyroid glands. Despite parathyroidectomy, the patient showed progressive skin necrosis and blistering over thighs, legs, feet, abdomen, buttocks and distal fingers (Figure 3). Wound dressing was carried out daily, without significant improvement.Figure 3 Dark brown-to-black necrotic plaques on the palmar aspect of the distal fingers.Download FigureDiscussionCalciphylaxis occurs mostly in patients with chronic renal failure and recent renal transplantation, although patients with normal renal function have been reported.2 Pre-dialysis patients and those undergoing both haemodialysis and peritoneal dialysis have presented with calciphylaxis.3 Kidney transplant recipients account for 32% to 38% of calciphylaxis cases, including patients with normally functioning grafts.4,5 Our patient had ESRD and was on haemodialysis after rejection of two kidney transplants. He received glucocorticoids and immunosuppressive medications, mycophenolate mofetil, tacrolimus, and ferrous sulphate. All are known precipitating factors for calciphylaxis.6,7Because of lowered vitamin D in ESRD patients (the active metabolite of vitamin D is transformed in the kidney), there is decreased calcium absorption from the intestine. Low serum vitamin D, low serum calcium, and high serum phosphate lead to secondary hyperparathyroidism. Most patients with calciphylaxis have abnormalities of calcium-phosphate homeostasis with elevated levels of parathyroid hormone, but these changes as well as an elevation of the calcium-phosporus product are not always present in this disease.5 This suggests that other important elements must contribute to calciphylaxis. Skin biopsy of the lesions showed numerous organized thrombi, which means a coagulation defect might be the missing link.8 Our patient had severe hyperparathyroidism.A pre-existing hypercoagulable status has also been hypothesized to predispose affected patients to calciphylaxis.8,9 In support of this mechanism, functional abnormalities in either protein C or protein S have been reported in several patients with calciphylaxis. However, these proteins have also been normal in other patients, including our case.4,5,10Early lesions of calciphylaxis manifest either as non-specific violaceous mottling, livedo reticularis, purpuric patches and plaques or as erythematous papules, plaques or nodules. More developed lesions have a stellate purpuric configuration with central cutaneous necrosis. Later lesions are exquisitely tender, extremely firm to touch, evolving into irregular ulcers covered by thick dark eschars. Lesions can be distributed proximally (on the thighs, buttocks and abdomen) or distally (below the knee). Our patient had proximal lesions initially, which later extended distally. Ninety percent of lesions are found on the lower extremities.1,11,12 Necrosis of the penis secondary to calciphylaxis has also been described.7,13Despite the dramatic cutaneous necrosis, no internal organ failure is reported in patients with calciphylaxis. Our patient’s cutaneous manifestations started as a very mild purpura over the thighs, which did not suggest calciphylaxis. Two weeks later they progressed to full-blown typical calciphylaxis lesions with bullae (which is a rare manifestation). Typical histologic findings are calcification within the media of small and medium-sized arterioles with extensive intimal hyperplasia and fibrosis. Subcutaneous calcium deposits with panniculitis and fat necrosis may sometimes be found. Frequently, vascular microthrombi are seen. Necrosis of overlying epidermis, and often surrounding dermis and subcutis is frequently seen.1,14 In our patient, typical histological findings were obvious.Treatment of calciphylaxis is largely supportive. Aggravating conditions should be addressed and trigger factors eliminated. Normalization of abnormal calcium and phosphorus levels by diet, phosphate binding agents, low-calcium hemodialysis and parathyroidectomy might help.1,15 Aggressive wound care and careful debridement of necrotic tissue are necessary to avoid wound infection and sepsis. Treatment of infections with appropriate antibiotics can be helpful.9 Treatment with hyperbaric oxygen16 and prednisone17 have been anecdotally reported.Patients with hypercoagulability may benefit from judicious anticoagulation. However, the role of anticoagulation in other cases of calciphylaxis is controversial. Prophylactic use of warfarin or heparin is probably not indicated since precipitation of calciphylaxis has occurred with indiscriminate use. In addition, most patients with ESRD have a prolonged bleeding time due to the uremic condition so anticoagulation may be contraindicated.8,18Total or subtotal parathyroidectomy with auto-transplantation is of therapeutic benefit to many but not all patients.1,19 Only few studies have been able to demonstrate a decrease in mortality rate in those patients who underwent parathyroidectomy.20,21 Normalization of calcium and phosphorus, continuous wound dressing, and total parathyroidectomy did not help our patient. Because of a normal coagulation profile and protein C and S levels, he was not a candidate for anticoagulation.The prognosis in patients with calciphylaxis is poor and the mortality rate is as high as 80%.4 In a review of 104 cases, Hotner et al found that 75% of patient with distal localization of lesions survived compared to 25% with proximal lesions.22 Unfortunately, most patients with calciphylaxis die of overwhelming infection and sepsis resulting from a compromised skin barrier in an immunosuppressed patient with chronic renal failure or after kidney transplantation. 14 No data exist regarding long-term survival, if one survives an episode of calciphylaxis.Two important and unique points about our case are the initial presentation with trivial purpuric lesions and the appearance of blisters, which is a rare manifestation of calciphylaxis. Fortunately, calciphylaxis is a rare disease. Its prevalence has not been established in our area, but is probably lower than estimations in other reports. Because of its generally aggressive behavior, and lack of effective treatment in most cases, early recognition of this disease is crucial. Our poor knowledge of the exact pathophysiology is still the limiting factor for intervention.ARTICLE REFERENCES:1. Oh DH, Eulan D, Tokugawa DA, et al. Five Cases of Calciphylaxis and a Review of the Literature . J Am Acad Dermatol. 1999; 40:979-987. Google Scholar2. Fader DJ, Kang S. Calciphylaxis Without Renal Failure . Arch Dermatol. 1996; 132:873-878. Google Scholar3. Fine A, Felming S, Leslie W. Calciphylaxis Presenting with Calf Pain and Plaques in 4 Continuos Ambulatory Peritoneal Dialysis Patients . Am Kidney Dis. 1995; 25:498-502. Google Scholar4. Hafner J, Keusch G, Wahl C, Sauter B, Urlimann A, Von Weizsacker F, et al. Uremic Small Artery Disease with Medial Calcification and Intimal Hyperplasia (so-called Calciphylaxis): A Complication of Chronic Renal Failure and Benefit from Parathyroidectomy . J Am Acad Dermatol. 1995; 33:954-962. Google Scholar5. Budisalijevic MN, Cheek D, Ploth DW. Calciphylaxis in chronic Renal Failure . J Am Soc Nephrol. 1996; 7:978-982. Google Scholar6. Selye H. Calciphylaxis. Chicago: University of Chicago Press; 1962. Google Scholar7. Ivker RA, Woosley J, Briggman RA. Calciphylaxis in Three patients with End-Stage Renal Disease . Arch Dermatol. 1995; 131:63-68. Google Scholar8. Mehta RL, Scott G, Sloand JA, et al. Skin Necrosis Associated with Acquired Protein C Deficiency in Patients with Renal Failure and Calciphylaxis . Am J Med. 1990; 88:252-257. Google Scholar9. Perez-Jijares R, Guzman-Zamudio JL, Payan-Lopez J, et al. Calciphylaxis in a Hemodialysis Patient: Functional Protein S Deficiency? Nephrol Dial Transplant. 1996; 11(9):1856-1859. Google Scholar10. Walsh JS, Fairly JA. Calciphylaxis . J Am Acad Dermatol. 1996; 35:786-787. Google Scholar11. Fisher AH, Morris DJ. Pathogenesis of Calciphylaxis: Study of Three Cases and Literature Review . Hum Pathol. 1995; 26:1055-1064. Google Scholar12. Walsh JS, Fairley JA. Calcifying Disorders of the Skin . J Am Acad Dermatol. 1995; 33:693-706. Google Scholar13. Wood JC, Monga M, Hellstrom WJG. Penile Calci[phylaxis] . Urology. 1997; 50:622-624. Google Scholar14. Requena L, Sanchez E. Panniculitis Part II. Mostly Lobular Panniculitis . J Am Acad Dermatol. 2001; 45:325-361. Google Scholar15. Lipsker D, Chosidow O, Martinez F, et al. Low-calcium Dialysis in Calciphylaxis . Arch Dermatol. 1997; 133:798-799. Google Scholar16. Vassa N, Twardpowski ZJ, Campbell J. Hyperbaric Oxygen Therapy in Calciphylaxis-Induced Skin Necrosis in a Peritoneal Dialysis Patient . Am J Kidney Dis. 1994; 23:878-881. Google Scholar17. Elamin Em, McDonad AB. Calcifying Panniculitis with Renal Failure: A New Management Approach . Dermatology. 1996; 192:156-159. Google Scholar18. Rudwaleit M, Schwarz A, Trautmann C. Severe Calciphylaxis in a Renal Patient on Long-Term Oral Anticoagulant Therapy . Am J Nephrol. 1996; 16(4):344-348. Google Scholar19. Ledbetter LS, Khoshnevis MR, Hsu S. Calciphylaxis . Cutis. 2000; 66:49-51. Google Scholar20. Dereure O, Lery H, Barneon G. Extensive Necrotizing Livedo Reticularis in a Patient with Chronic Renal Failure, Hyperparathyroidism and Coagulation Disorder: Regression After Subtotal Parathyroidectomy . Dermatology. 1996; 2:167-170. Google Scholar21. Massry SG, Gordon A, Coburn JW. Vascular Calcification and Peripheral Necrosis in a Renal Transplant Recipient. Reversal of Lesions Following Subtotal Parathyroidectomy . Am J Med. 1970Sep 49(3):416-422. Google Scholar22. Hafner J, Kensch G, Wahl C, et al. Calciphylaxis: A Syndrome of Skin Necrosis and Acral Gangrene in Chronic Renal Failure . Vasa. 1998; 27:137-143. Google Scholar Previous article Next article FiguresReferencesRelatedDetails Volume 25, Issue 5September-October 2005 Metrics History Accepted1 May 2004Published online6 October 2005 AcknowledgementsI gratefully acknowledge Dr. Marwan Al Khawajah, Professor and Chairman of Dermatology, and Dr. Nauman Tareef, Assistant Professor and Consultant Nephrologist, King Saud University, College of Medicine, Riyadh, Saudi Arabia for their help by reviewing the manuscript; and Ms. Alma Bautista for typing this manuscript.InformationCopyright © 2005, Annals of Saudi MedicineThis work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.PDF download" @default.
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