Baylor University Medical Center Proceedings

The peer-reviewed journal of Baylor Scott & White Health

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Metastatic Calcinosis Cutis in End-Stage Renal

Disease

Victoria A. Jaeger, Megan G. Newman & Curtis R. Mirkes

To cite this article: Victoria A. Jaeger, Megan G. Newman & Curtis R. Mirkes (2017) Metastatic
Calcinosis Cutis in End-Stage Renal Disease, Baylor University Medical Center Proceedings, 30:3,
368-369, DOI: 10.1080/08998280.2017.11929652

To link to this article: https://doi.org/10.1080/08998280.2017.11929652

Published online: 11 Dec 2017.

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Metastatic calcinosis cutis in end-stage renal disease
Victoria A. Jaeger, DO, Megan G. Newman, MD, and Curtis R. Mirkes, DO
Alterations in calcium and phosphorus levels and joint pain are a com-
mon occurrence in end-stage renal disease patients. However, metastatic
calcinosis cutis is a rare diagnosis that often combines these two findings,
with extensive soft tissue calcification surrounding a large joint being
the hallmark of this disease. The exact mechanism behind this clinical
entity is unknown. The treatment and complications can be severe and
disabling. Here, we discuss the case of a 26-year-old man presenting
with unusually advanced skin and joint calcification of the shoulders,
neck, hand, and penis.
C
alcinosis cutis is an unusual disorder characterized by
large calcium phosphate deposition into cutaneous and
subcutaneous tissues. Five subtypes of calcinosis cutis
exist: dystrophic, metastatic, idiopathic, iatrogenic, and
calciphylaxis. The shoulders, elbows, and hips are typically the
joints affected by these lesions. However, cases have been re-
ported in various other periarticular areas of the body (1). Pa-
tients present with symptoms such as pain, joint stiffness, nerve
compression, inflammation, fistula formation, infection, and
sometimes systemic symptoms such as fever (1, 2). Presenting
skin findings may be nodules with extrusion of a chalky while
substance. Here, we describe a patient who presented with ad-
vanced metastatic calcinosis cutis with multiple lesions of the
large joints, neck, hands, and penis.
CASE REPORT
A 26-year-old man with a history of focal segmental glo-
merulosclerosis and a congenital solitary kidney presented with
left hand pain and fatigue. He had been treated for presumed
gout affecting the left hand 12 days prior to presentation at the
emergency department without resolution of his symptoms.
He appeared cachectic and ill, with multiple areas of calcium
deposits on the left hand, left shoulder, right axilla (Figure 1),
and penis. The left third digit was swollen with calcific deposits
and nodules. The dorsal and left lateral penile shaft had hard,
nontender nodules without swelling. Initial laboratory values
were a calcium level of 9.7 mg/dL, phosphorus level of 10.6 mg/
dL, parathyroid hormone level of 5646 pg/mL, creatinine of
7.60 mg/dL, blood urea nitrogen of 124 mg/dL, and estimated
glomerular filtration rate of 8.8 mL/min/1.73 m2. Computed
368
tomography without intravenous contrast showed extensive,
lobulated soft tissue calcifications in both shoulders and the
left chest wall, extending into the neck (Figure 2). Findings
were consistent with metastatic calcinosis cutis secondary to
chronic kidney disease stage 5. Hemodialysis, cinacalcet, a renal
diet, and phosphate binders were started. A calcium-phosphate
product <50 was targeted. Sodium thiosulfate was given follow-
ing dialysis, and a subtotal parathyroidectomy was ultimately
performed to resolve tertiary hyperparathyroidism.
DISCUSSION
Hundreds of cases of calcinosis cutis have been reported,
but only a small number have involved areas of the hand. The
typical sites of involvement are the superior and lateral shoulder,
posterior elbows, and lateral hip and gluteal regions (1). Other
areas such as hands, feet, spine, temporomandibular joint, and
knee have been observed. Our patient had extensive involve-
ment of the usual shoulder regions, but also involvement of the
digits, neck, and genitals.
Five subtypes of calcinosis cutis exist. Our patient presented
with the subclass termed metastatic calcinosis cutis, which was
likely secondary to end-stage renal disease. Though rare, calci-
nosis cutis is a severe complication of chronic kidney disease
with an incidence of 0.5% to 3% (2). The subclass of calcinosis
cutis that our patient presented with is characterized by abnor-
mal phosphorus or calcium metabolism resulting in widespread
deposition of calcium in the skin and soft tissues (3). Meta-
static calcinosis cutis has been linked to other conditions such
as sarcoidosis, Albright hereditary osteodystrophy, neoplasms,
and hypervitaminosis D (3). The most common predisposing
condition, however, is chronic kidney disease. A mechanism of
pathogenesis of calcinosis cutis has been proposed. It is believed
that repeated small trauma occurs, which creates hemorrhages
in tissues surrounding the joints and initiates an inflammatory
response (1, 4).
From the Department of Internal Medicine, Baylor Scott and White Hospital and
Texas A&M Health Science Center College of Medicine, Temple, Texas.
Corresponding author: Megan G. Newman, MD, Department of Internal
Medicine, Baylor Scott and White Hospital, Texas A&M Health Science Center
College of Medicine, 2401 S. 31st Street, Temple, TX 76508 (e-mail: Megan.
Newman@BSWHealth.org).
Proc (Bayl Univ Med Cent) 2017;30(3):368–369
 a b (1). CT is typically
used to evaluate the
disease burden for
surgical planning.
Another key feature
is absence of erosion
and destruction of
nearby bone (1).
Laboratory studies
should be performed
to rule out other dis-
ease processes before
a definitive diagnosis
of calcinosis cutis is
made, as it is a diag-
nosis of exclusion (1,
4). If the diagnosis
remains uncertain,
biopsy can be per-
Figure 1. Extensive ulcerated calcific deposits in (a) the left third digit and (b) the left axilla. formed (1).
Secondary cal-
Metastatic calcinosis cutis usually presents with benign nod- cinosis cutis is usually treated with medical management of
ules at periarticular sites (3). However, symptoms such as pain, the underlying cause. When medical therapy has failed, sub-
joint stiffness, nerve compression, inflammation, fistula forma- total parathyroidectomy is indicated. Renal transplant is also
tion, and infection may be present at the time of diagnosis (1, 2). an option in secondary hyperparathyroidism, as it resolves the
Physical exam findings may be significant for decreased range of underlying process (5), but may not be an option for tertiary
motion or pain at the affected site. Our patient presented with hyperparathyroidism, as in our case. Surgical debridement of
advanced disease with large, calcific ulcerated skin lesions. In the lesions is indicated for recurrent infection, ulceration, and
metastatic calcinosis cutis, calcification is typically seen in blood pain or impairment (1). Treatment includes restricting calcium
vessels, kidneys, lungs, and gastric mucosa as well (3). Radio- and phosphorus intake (to <700 mg/day) in the diet and the use
graphic evidence of the disease is the primary modality of di- of phosphate binders (1, 2, 6, 7). Other medical therapies such
agnosis, along with supporting laboratory data. The appearance as vinpocetine, sodium thiosulfate, and intravenous pamidro-
on computed tomography (CT) is characterized as cystic loculi nate have been used with variable success (1). In our patient,
with fluid and calcium layering known as the sedimentation sign sodium thiosulfate was used. This drug was originally used to
treat cyanide poisoning, but has shown efficacy in several cases
of calcinosis cutis (2, 6).

1. Fathi I, Sakr M. Review of tumoral calcinosis: a rare clinico-pathological

entity. World J Clin Cases 2014;2(9):409–414.
2. Malbos S, Urena-Torres P, Cohen-Solal M, Trout H, Liote F, Bardin T,
Ea HK. Sodium thiosulphate treatment of uraemic tumoral calcinosis.
Rheumatology 2014;53(3):547–551.
3. Fernandez KH, Ward DS. Calcinosis cutis: etiology and patient evaluation.
In Post TW, ed. UpToDate. Waltham, MA: UpToDate (accessed February
24, 2017).
4. Stock H. Tumoral calcinosis and its mimics. Conn Med 2014;78(8):497–
499.
5. Takashima S, Sakamoto T, Ota M. Tumoral calcinosis in chronic renal
failure. Lancet Diabetes Endocrinol 2014;2(10):852.
6. Jost J, Bahans C, Courbebaisse M, Tran TA, Linglart A, Benistan K, Lien-
hardt A, Mutar H, Pfender E, Ratsimbazafy V, Guigonis V. Topical sodium
thiosulfate: a treatment for calcifications in hyperphosphatemic familial
tumoral calcinosis? J Clin Endocrinol Metab 2016;101(7):2810–2815.
7. Xie T, Zhang Y, Tang X, Chen M, Zhang W. Amelioration of uremic
Figure 2. CT scan showing extensive, lobulated soft tissue calcification about the
tumoral calcinosis with high-flux hemodialysis treatment. Int Urol Nephrol
left shoulder girdle and left chest wall in aggregate measuring up to 16.1 cm an-
2014;46(7):1467–1468.
teroposteriorly × 17.6 cm transversely × 11.5 cm in the craniocaudal dimension.

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