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INDIAN JOURNAL OF KIDNEY DISEASES
38
Anupama Y J
Address for correspondence:
Dr Anupama Y J, Senior Consultant Nephrologist
Nanjappa Hospitals, Shivamogga 577201, Karnataka
E: anupamayj@ gmail.com
IMAGES IN NEPHROLOGY
Calciphylaxis
Figure 1: Clinical photograph of the Skin lesions
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Volume-1, Issue-1 Jan-March 2022 IJKD 39
A 55-year old gentleman with diabetic chronic
kidney disease on regular haemodialysis for the
past three years, sought advice for painful skin
lesions on the left leg. There was no history of fever,
trauma or insect bite. His other comorbidities were
obesity, ischemic heart disease, hypertension,
HCV seropositive status and left subclavian
vein stenosis with severe venous hypertension.
His medications included nifedipine, clonidine,
sodium bicarbonate, periodic erythropoietin and
iron sucrose injections. On evaluation, he had a
2x4 cm necrotic ulcer covered with eschar over
the left leg two inches above the lateral malleolus
and two smaller necrotic areas 0.5x0.5 cm above
the medial malleolus (Figure 1). Dorsalis pedis
and posterior tibial arterial pulsations were not
felt in both legs. His lab results were as follows:
Haemoglobin-7.4 g/dl, serum creatinine-10.5mg/
dl, calcium-10.5 mg/dl, phosphorus - 4.1 mg/
dl (Ca*P product 41.1mg2 / dl2), albumin - 4.1 g/
dl, alkaline phosphatase-163 IU/l, parathyroid
hormone -276.8 pg/ml(normal-10-65 pg/ml).
X-ray of the left foot showed extensive calcification
of the blood vessels. Histopathologic examination
of skin biopsy revealed extensive calcification in
the subcutaneous tissue with calcific plugs in the
Figure 2: Skin biopsy of the lesion demonstrating calciphylaxis(HE, 40x)
1. Stratified squamous epithelium
2. Calcification in the subcutis
3. Calcification in the blood vessels
4. Calcification of the walls along with calcific obliteration of the lumen
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INDIAN JOURNAL OF KIDNEY DISEASES
40
dermal vessels confirming calciphylaxis (Figure
2). He was treated with intensification of dialysis
against low calcium concentrates, cinacalcet and
antibiotics. Sodium thiosulfate was not available
and not given. The lesions worsened despite
these measures. He succumbed to intractable
sepsis ten weeks later.
Calciphylaxis or calcific uraemic
arteriolopathy(CUA) is a chronic progressive
syndrome of arteriolar media calcification,
thrombotic ischemia, and necrotic ulceration.1
It is mostly seen in patients with advanced
chronic kidney disease on haemodialysis. It may
rarely be seen in renal transplant recipients with
functioning kidneys.2,3 Few cases are reported
even in non-uraemic settings such as alcoholic
liver disease, connective tissue disease and
malignancies.3 The pathogenesis is unclear.
Disturbances in calcium-phosphorus metabolism
are mostly implicated. Hyperparathyroidism,
increased serum phosphorus, increased calcium
x phosphorus product>70 mg2/dl 2 , female sex,
obesity, diabetes, vitamin D therapy and warfarin
use are some of the factors associated with
calciphylaxis.1,4 Lesions start with tender red areas
developing into indurated plaques or nodules.
Patients may subsequently develop an eschar
followed by frank ulceration, gangrene or sepsis.
Proximal pattern of involvement with lesions
abdomen, thigh and buttocks carries a worse
prognosis.5 Rarely, penis, breasts and ear lobules
may become necrotic. The treatment is not well-
defined. Parathyroidectomy, oral calcimimetics,
sodium thiosulfate are some of the measures
that have been employed with variable success.
Aggressive wound debridement has been
advocated and hyperbaric oxygen may be useful
in healing of ulcers.5,6 The condition is associated
with significant morbidity and mortality. 80% of
the affected patients die of sepsis or organ failure,
usually within a year.1 Our patient was obese and
diabetic, but parathyroid hormone levels were
within acceptable range for the level of kidney
function. His calcium phosphorus product was
also within the recommended range for dialysis
patients. The cause for the lesions was not clear.
High index of suspicion in patients with CKD is
necessary for early diagnosis and limitation of
morbidity.
References:
1. Wollina U. Update on cutaneous calciphylaxis.
Indian J Dermatol 2013;58:87-92
2. Urmila A, Kumar R, Rao V. Secondary
Subcutaneous Rhizopus Infection in a
Posttransplant Recipient with Calcific Uremic
Arteriolopathy. Case Reports in Infectious
Diseases, vol. 2020, Article ID 8695204.
https://doi.org/10.1155/2020/8695204
3. Nigwekar SU, Wolf M, Sterns RH, Hix
JK.Calciphylaxis from nonuremic causes: a
systematic review.Clin J Am Soc Nephrol.
2008 Jul; 3(4):1139-43
4. Nigwekar SU, Kroshinksy D, Nazarian RM, et
al. Calciphylaxis: Risk Factors, Diagnosis, and
Treatment. Am J Kidney Dis 2015;66(1):133-
146. doi:10.1053/j.ajkd.2015.01.034.
5. Brandenburg VM, Kramann R, Rothe H,
Kaesler N, Korbiel J, Specht P et al.Calcific
uraemic arteriolopathy (calciphylaxis): data
from a large nationwide registry.Nephrol Dial
Transplant. 2017 Jan 1;32(1):126-132.
6. Bhambri.A, Del Rosso JQ. Calciphylaxis. J
Clin Aesthet Dermatol. 2008;1(2):38-41
Anupama Y J : Calciphylaxis
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