Temporal artery calciphylaxis has rarely been described in chronic kidney disease patients on dialysis. We report a case of 72-year-old Caucasian man with multiple comorbidities and end-stage renal disease on dialysis who presented with temporal artery calcification leading to bilateral loss of vision followed by extensive skin lesions including one on glans penis. While on peritoneal dialysis, he developed anterior ischemic optic neuropathy, had no improvement on high dose steroids, and temporal artery biopsy showed marked calcification without any evidence of vasculitis. Few weeks later on hemodialysis, he developed widespread cutaneous lesions on extremities and penile necrosis with skin biopsy revealing calciphylaxis. On literature review of calciphylaxis in chronic kidney disease, we found only four cases of temporal artery calciphylaxis leading to anterior ischemic optic neuropathy and blindness. We believe this is the first case in which the rare temporal artery calciphylaxis and the uncommon penile necrosis are being described together. The objective is to emphasize the need to recognize this condition early in the CKD patients on dialysis presenting with visual symptoms as the different treatment strategies may help prevent complete loss of vision and also modify or prevent a full blown calciphylaxis

A Haase-Fielitz

A K Saxena

A Papagianni

Francisco

Manzoor A Shah

Michael W Roppolo

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Hindawi Publishing Corporation
Case Reports in Nephrology
Volume 2012, Article ID 309727, 4 pages
doi:10.1155/2012/309727
Case Report
Calciphylaxis: Temporal Artery Calcification Preceding
Widespread Skin Lesions and Penile Necrosis
Manzoor A. Shah and Michael W. Roppolo
Division of Internal Medicine, Baton Rouge General Hospital, 3600 Florida Boulevard, Baton Rouge, LA 70806, USA
Correspondence should be addressed to Manzoor A. Shah, manzoor.shah@brgeneral.org
Received 27 March 2012; Accepted 16 May 2012
Academic Editors: A. L. de Francisco, A. Haase-Fielitz, A. Papagianni, and A. K. Saxena
Copyright © 2012 M. A. Shah and M. W. Roppolo. This is an open access article distributed under the Creative Commons
Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is
properly cited.
Temporal artery calciphylaxis has rarely been described in chronic kidney disease patients on dialysis. We report a case of 72-
year-old Caucasian man with multiple comorbidities and end-stage renal disease on dialysis who presented with temporal artery
calcification leading to bilateral loss of vision followed by extensive skin lesions including one on glans penis. While on peritoneal
dialysis, he developed anterior ischemic optic neuropathy, had no improvement on high dose steroids, and temporal artery
biopsy showed marked calcification without any evidence of vasculitis. Few weeks later on hemodialysis, he developed widespread
cutaneous lesions on extremities and penile necrosis with skin biopsy revealing calciphylaxis. On literature review of calciphylaxis
in chronic kidney disease, we found only four cases of temporal artery calciphylaxis leading to anterior ischemic optic neuropathy
and blindness. We believe this is the first case in which the rare temporal artery calciphylaxis and the uncommon penile necrosis are
being described together. The objective is to emphasize the need to recognize this condition early in the CKD patients on dialysis
presenting with visual symptoms as the different treatment strategies may help prevent complete loss of vision and also modify or
prevent a full blown calciphylaxis.
1. Introduction
Calciphylaxis or calcific uremic arteriolopathy (CUA) is a
rare condition that occurs in chronic kidney disease (CKD)
stage 4 or 5 with secondary hyperparathyroidism though it
has been described in few nonrenal conditions as well. With
pathogenesis poorly understood it has high morbidity and
a 6-month mortality rate of about 80% [1, 2]. It occurs in
1–4% of dialysis patients usually involving areas of lower
extremities with high adipose tissue like thighs and buttocks
[3]. Lesions appear as violaceous painful indurated nodules,
plaques, necrotic eschars, ulcerations, and dry gangrene.
Diagnosis is based on clinical signs and symptoms and
biopsy of the lesion by demonstrating calcification in the
vascular media, intimal hyperplasia, inflammation, oblit-
erative endovascular fibrosis and microthrombi in small-
and medium-sized vessels of skin, and subcutaneous tissue
leading to necrosis of dermal, subdermal, and adipose tissue
[4].
2. Case Report
A 72-year-Caucasian man was referred to our Long Term
Acute Care (LTAC) Hospital for continuation of hemodial-
ysis and aggressive wound care. He had a PMH of hyper-
tension and diabetes mellitus diagnosed at the age of 59
when he had aortic valve replacement (AVR) with a St Jude’s
valve for aortic stenosis and had been on coumadin since
then. He also had atrial fibrillation (AF) since 2005 followed
few years later by congestive heart failure (CHF) with right-
sided heart failure being more pronounced than the left,
severe tricuspid regurgitation, pulmonary artery pressure of
68 mm Hg, concentric left ventricular hypertrophy, and a
LVEF of 53%. Also had diabetic complications of background
retinopathy and nephropathy with stable Creatinine (Cr
∼1.7 mg/dL) for several years. In July 2011, the patient
started having problems with volume overload with swelling
of lower extremities and ascites. His cardiologist tried
outpatient diuresis with metolazone and bumetanide while
2 Case Reports in Nephrology
balancing it with his degree of CKD but not with much
success. Two months later, he needed to be admitted to
the hospital with worsening volume overload, weeping leg
edema, massive ascites, and declining renal functions with
a BUN/Creatinine of 97/2.0 mg/dL. Diuretics were used
intravenously without much effect again. He was planned
for continuous ambulatory peritoneal dialysis (CAPD) and
had a Tenkchoff PD Catheter put in laproscopically. In the
meantime, hemodialysis was given for three weeks before
starting CAPD at low volume exchanges and 5 times per
day at his home. His medications included bumetanide,
cholecalciferol, calcium acetate, coumadin, iron, and lantus.
In December 2011, he developed sudden loss of vision in his
left eye. His ophthalmologist found a left optic disc edema,
venous dilatation, choroidal hypoperfusion, and a decreased
visual acuity (VA) of 20/200 in his left eye and 20/40 in
his right eye. He was diagnosed to have anterior ischemic
optic neuropathy (AION). Three days later, he developed
decreased vision in the right eye with VA of 20/80. Both
optic discs were edematous with left being more than the
right. The patient was started on high dose steroids and
admitted to the hospital for temporal artery biopsy which
revealed large coarse marked calcifications in the muscular
artery with no evidence of vasculitis. Steroids did not make
any improvement and were subsequently stopped. Next eye
exam a week later showed a VA of 20/200 in both eyes due to
AION. During the hospital stay, his peritoneal dialysis (PD)
volume exchanges were increased to 2 liters per exchange.
Four weeks later, he was again admitted to the
hospital for falls, and worsening uremia. His lab work
showed a BUN 69 mg/dL, creat 6.3 mg/dL, GFR 9, cal-
cium 7.9 mg/dL, phosphorus 6.6 mg/dL, calcium phosphate
product 51.48 mg/dL, parathormone (PTH) 164 pg/mL, ALP
636 IU/L, Hb 10.1 g/dl, and Hct 30.2%. He had been
noncompliant with PD exchanges doing only a couple of
exchanges per day. Hemodialysis (HD) was started through
a left IJ Vascath, and a right brachiocephalic AV fistula
was created. He developed fiercely spreading wide areas of
indurated skin plaques, woody in feel and dusky in color at
multiple sites on thighs, legs, arms, and glans penis with two
small black eschars on thighs. After he was stabilized on HD,
he was transferred to LTAC for continuation of hemodialysis
and aggressive wound care. He had debridement of left
thigh wound, and biopsies were taken from both thighs
which revealed calciphylaxis with prominent calcification of
the small vessels in the subcutaneous fat accompanied by
ischemic necrosis and ulceration of the overlying epidermis.
The patient developed sepsis with low-grade fever, high
WBC count, neutrophilia, and altered mental status. Urinal-
ysis was normal, blood cultures were negative but wound
culture grew methicillin sensitive Staphylococcus aureus
(MSSA), and antibiotics were given per sensitivity. His X-ray
chest showed pleural thickening and calcification just above
left hemidiaphragm and X-rays of extremities showed exten-
sive vascular calcification (Figure 1).
Serum PTH increased to 374 pg/mL with vitamin D
<8.0 pg/mL. Hemodialysis frequency per week was increased,
and the duration per session was increased to four hours.
Figure 1
Figure 2
Calcium acetate was stopped and replaced by sevelamer—a
noncalcium phosphate binder. He continued his other med-
ications including Cholecalciferol and Coumadin. Wound
care was carried out by a physician-led dedicated wound care
team. After debridement the wound on the left thigh refused
to heal with even further blackening of wound margins. Anti-
biotics were broadened to cover gram positive cocci, gram
negative rods and anaerobes for continuing sepsis. However,
his calciphylaxis worsened with black eschars developing
over wide areas of indurated skin lesions and glans penis
becoming gangrenous (Figures 2 and 3).
Clinically, the patient took another turn for worse when
he developed coagulopathy with prolonged INR and malena.
With unabating calciphylaxis, nonhealing wounds, sep-
sis, coagulopathy, and gastrointestinal bleeding the patient
died within ten weeks of presentation with temporal artery
calciphylaxis causing blindness and six weeks of widespread
skin lesions.
Case Reports in Nephrology 3
Figure 3
3. Discussion
Treatment of calcific uremic arteriolopathy is often unsuc-
cessful due to pathogenesis being unclear and diagnosis
frequently delayed or missed. With increasing number of sur-
viving patients on dialysis and high prevalence of CKD, more
cases of calciphylaxis are being recognized now. Risk factors
include high serum phosphate level with increased calcium
phosphate product, secondary hyperparathyroidism, and use
of calcium containing phosphate binders, use of calcitriol,
diabetes mellitus, obesity, low albumin, Coumadin therapy,
female gender, and Caucasian race [2, 5].
In our case, noncompliance with peritoneal dialysis most
likely led to hyperphosphatemia with a modest increase in
CaP04 product and secondary hyperparathyroidism. When
the patient first presented with temporal artery calcification
leading to AION, the diagnosis of calciphylaxis was not con-
sidered because of this rare complication occurring in isola-
tion without skin lesions. However, few weeks later it became
apparent when the widespread skin lesions appeared like a
wildfire with unrelenting course leading to cutaneous eschars
and penile gangrene with skin biopsy confirming the diag-
nosis. Coumadin therapy most likely rendered additional
risk to our patient and probably aggravated calciphylaxis.
Coumadin in animals has been shown to induce vascular
calcification by preventing activation of matrix gla protein
(MGP) in the vessel wall. MGP is a vitamin K dependent pro-
tein, which becomes biologically active after carboxylation
and prevents vascular calcification. However, the relationship
of Coumadin to vascular calcification in humans is not
fully known [6, 7]. Considering the benefit/risk ratio of
anticoagulation in our patient with AVR, Coumadin was
continued.
Cornerstone of therapy is to keep serum phosphate
<6 mg/dL and a CaPo4 product <55 mg/dL which we
obtained by changing calcium-based phosphate binder to
sevelamer and performing hemodialysis with a low-calcium
dialysate [8]. Debridement of the wound in calciphylaxis is
controversial and generally not advisable and in our case the
debrided wound refused to heal. On account of his advanced
debility patient was not considered for parathyroidectomy.
Pharmacotherapeutic interventions like sodium thiosulfate
therapy, hyperbaric oxygen therapy or cinacalcet were not
used due to limited anecdotal evidence and his moribund
condition [9, 10]. Sodium thiosulfate a potent antioxidant
that also increases the solubility of calcium deposits, has been
reported to lead to marked improvement of calciphylaxis
[11]. Hyperbaric oxygen therapy has been used with some
success in calciphylaxis with local tissue hypoxia in wounds.
It restores tissue oxygen to normal or above normal level
thereby enhancing collagen production, fibroblast prolifer-
ation, and angiogenesis [12].
Calciphylaxis of temporal artery in ESRD leading to
nonarteritic AION and blindness has rarely been described
and on review of literature we found only 4 cases reported
[13–16]. Penile necrosis due to calciphylaxis is quite uncom-
mon in ESRD as penis has a rich blood supply from its dorsal
and deep arteries and also from urethral artery [17–19]. Its
occurrence in the context of ESRD, diabetes, and dialysis
carries a high mortality. We believe this is the first case
of CUA in whom temporal artery calciphylaxis and penile
necrosis are being reported together.
Temporal artery calciphylaxis in CKD patients on dialysis
presenting with symptoms of visual loss needs to be rec-
ognized early as the different treatment strategies may help
prevent complete loss of vision and also modify or prevent
a full blown calciphylaxis—a condition known to have very
high morbidity and mortality.
Conflict of Interests
The authors declare that this paper is not influenced by sec-
ondary interests such as financial gains.
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Case Report Calciphylaxis: Temporal Artery Calcification Preceding Widespread Skin Lesions and Penile Necrosis

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Case Report Calciphylaxis: Temporal Artery Calcification Preceding Widespread Skin Lesions and Penile Necrosis

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