Download Danielle N. Butto DPM, AACFAS , Melissa Gulosh DPM

Danielle N. Butto DPM, AACFAS1 , Melissa Gulosh DPM, PGY-32 , Jeffrey Martone DPM, FACFAS2
1Fellow,
Statement of Purpose
Case review of a case of calciphylalxis with the first
presenting lesion on the heel. The patient was evaluated
initially and found to have no heel lesions. Six days after
initial evaluation, the service was re-consulted for a pressure
ulceration to heel which was found to be calciphylaxis. This
study serves to review calciphylaxis and its characteristics.
Literature Review
It is estimated that calciphylaxis occurs in 1-4% of dialysis
patients each year with a female preponderance of 3:1.1,2 It is
more common in Caucasian females.3 Painful, violaceous
mottled skin lesions that progress to plaque and/or nodules are
the first dermatologic changes. The lesions progress to nonhealing ulcerations that ultimately develop eschar formation
and gangrene.4 Calciphylaxis is seen in the lower extremities
in 90% of cases.2 Sepsis from infected ulcers is a frequent
cause of death. Peripheral pulses are often preserved and favor
the diagnosis of calciphylaxis.1
Laboratory findings typically include increased parathyroid
hormone levels, seen in 80% of patients, and elevated calcium
and phosphorus concentrations2. Calcium-phosphorus
product levels greater than 70 mg/dl favors the diagnosis of
calciphylaxis.1 Elevated levels of alkaline phosphatase and
low serum albumin are also frequently observed.
Radiographic evaluation may demonstrate pipe-stem pattern
of vascular calcification.3 Less concrete associations have
been made between low protein C and S levels as well5. A
high index of suspicion is critical for making the diagnosis of
calciphylaxis especially in light of normal lab values. Risk
factors include end stage renal disease, long term dialysis,
female gender, obesity (BMI >30), presence of autoimmune
disorder, and a hypercoagulable state. In addition, the
administration of certain medications is associated with an
increased risk of developing calciphylaxis. These medications
include warfarin, systemic corticosteroids and high dose
vitamin D6.
Ankle and Foot Care Centers, Youngstown OH, 2 St Francis Hospital and Medical Center, Hartford CT
Case Study
Case Study
Analysis and Discussion
A 61y Hispanic female presented to ED, from a skilled
nursing facility, with a temperature of 102.5⁰F with
complaints of thigh redness and swelling, abdominal
pain, nausea, and vomiting. Past medical history was
significant for asthma, congestive heart failure,
hypertension, gastroesophageal reflux disease, chronic
renal failure (dialysis started Aug 2012), diabetes
mellitus, rheumatoid arthritis (on chronic steroids),
chronic leukocytosis, depression, anxiety, and a history
of an embolic CVA (on Coumadin). Past surgical
history was significant for I&D of posterior right thigh
abscess and bilateral total knee replacements. She had
no history of smoking, alcohol, or intravenous
substance abuse. Her white count on admission was
31.1. Patient was admitted with the diagnoses with
urinary tract infection, cholelithiasis, questionable
BONE SCAN FINDINGS
clostridium difficile, and bilateral lower extremity
cellulitis. Antibiotics were initiated to include oral
Vancomycin, intravenous Flagyl, and intravenous
Ancef. Further lab work performed on the floor
revealed an albumin of 2.0 (normal range 3.5-5.0), prealbumin 8.9 (normal range 17-42), fibrinogen 367
(normal range 204-431), phosphorus 1.9 mg/dl (normal
range 2.5-4.5), alkaline phosphatase 313 U/L (normal
range 38-126).
Podiatry was consulted on hospital day 10. Physical
examination revealed palpable dorsalis pedis and
HISTOLOGIC
FINDINGS
posterior tibial
pulses bilateral.
A superficial ulcer was
noted at the calcaneal-cuboid joint of the left foot with
no purulence, no malodor, and no probing. Patient was
also noted to have a right posterior leg superficial
ulceration with no purulence, no malodor, and no
probing. No erythema was noted to either lower
extremity at this time. X-Ray imaging showed
evidence of previous Charcot of the left foot without
periosteal reaction and no evidence of osteomyelitis.
Continued
The hallmark signs of calciphylaxis must be
kept in mind when assessing the patient.
Affected patients will usually have intact
peripheral pulses, bilateral necrosis, and
frequent involvement of the lower extremity6
The clinical manifestations of calciphylaxis are
similar to those of other vascular disorders
therefore making diagnosis difficult. Disorders
that share the characteristics of calciphylaxis
include:Arteriosclerosis, DIC, Coumadin
necrosis, connective tissue disorders, CREST
and Scleroderma. Arteriosclerosis/cholesterol
embolus is a disease process of the mediumlarge arteries rather than the small-medium.
Necrosis is generally seen at the tips of the
digits. Unlike vasculitis, there are very few
inflammatory cells in and around the blood
7
.vessels unless the skin is secondarily infected
Disseminated intravascular coagulation presents
similar to a deep vein thrombosis with persistent
bleeding from puncture sites.
Scleroderma/CREST patients typically have
Raynaud’s phenomenon along with swelling of
the distal extremities and polyarthralgia.
Hematology/Oncology was consulted with
recommendations that stated the patient had a vitamin
D deficiency secondary to malabsorption/diarrhea, as
well as malnutrition. Treatment included vitamin D
therapy. Serology for vasculitis was negative
Six days after the initial podiatric evaluation, the
patient had developed a left posterior heel ulceration
with periwound erythema and a necrotic base. Reexamination of the extremities two days later revealed
violaceous tissue with patches of necrosis to bilateral
legs. Further laboratory work was ordered showing
normal calcium (8.2), normal phosphorus (3.7) and
normal parathyroid hormone (56.9). A biopsy was
performed which revealed “Multifocal calcium
deposition, including calcification of small and
medium size vascular structures with ischemic change.
Histologic findings consistent with calciphylaxis”. The
patient was started on Sodium Thiosulfate 25g IV
MWF.
CT SCAN FINDINGS
Analysis and Discussion
Discussion and Conclusion
Continued
The gold standard for diagnosis of calciphylaxis is
biopsy. There is no pathognomonic feature of
calciphylaxis on histologic examination; however,
the most consistent microscopic feature is acute and
chronic panniculitis with a predominantly septal
pattern. In addition, micro-calcifications in small to
medium sized venules are appreciated8. A high
index of suspicion is required when diagnosing and
treating calciphylaxis. The early stages of the
disease often mimic other disease processes. This
particular case was complicated by the presenting
lesions location of the heel. The patient was thought
to have a decubitus ulceration. It was not until a
review of the patient’s notes that it was noted she
was lesion free on admission. In addition, the
patient’s laboratory results were within normal
limits. The patient’s co-morbidities along with the
rapid progression of necrosis led to the decision to
biopsy. Initiation of treatment in a timely fashion
resulted in preservation of the patient’s limb, while
preventing sepsis.
.
References
1.
Figure 1: Initial evaluation of L heel and R lower extremity
Figure 3: Histopathology of biopsy with calcifications in adipose
Figure 2: L heel, L lower extremity and R lower extremity 6 days after
initial evaluation
Figure 4: L & R Lower Extremity after treatment w Sodium Thiosulfate
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