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CALCIPHYLAXIS WITH PSEUDOXANTHOMA ELASTICUM-LIKE CHANGES
DR. KARA HOVERSON, M.D. PGY-2, NATIONAL CAPITAL CONSORTIUM DERMATOLOGY RESIDENCY
DR. WENDI WOHLTMANN, M.D DERMATOPATHOLOGIST, WILFORD HALL AMBULATORY SURGICAL CENTER
B ACKGROUND
• Calciphylaxis is a progressive vascular calcification with
associated ischemic necrosis.
• Calcification triggers intimal proliferation and narrows
the arterioles resulting in necrotic skin lesions that are
resistant to healing and prone to infections.
• This is a serious condition with an overall mortality rate
of 80-85%.
C ASE
• 74 year old female with past medical history significant
for diabetes mellitus, obesity, atrial fibrillation,
hyperlipidemia, COPD, congestive heart failure and B
cell lymphoma presented with painful plaques on the
bilateral thighs. No recent travel.
• Labs were significant for persistently elevated BUN,
but normal Cr and albumin. PTH and Ca were also
within normal limits.
H&E, 100x Calcium deposition in
the wall of small to medium sized
blood vessels along with thickened
fibrous septae.
H&E, 200x Thickened fibrous
septa with "bramble bush"
appearing elastic fibers (PXElike changes).
D ISCUSSION
• Calciphylaxis with PXE-like changes is a rare
condition, with two reported cases to date. Both
cases were in patients with end stage renal disease.
• Risk factors for calciphylaxis in our patient included
DM, obesity and warfarin use. Prothrombic states
have been found to be important risk factor.
• Warfarin was discontinued and she was treated
with IV sodium thiosulfate and hyperbaric oxygen.
She responded well initially but was hospitalized for
CHF seven months later, with worsening of
condition and subsequent death.
Slightly indurated reticulated erythematous plaques on the
bilateral thighs, some with central duskiness and ulcerations with
clean moist bases.
REFERENCES
1. Weenig RH, Sewell LD, Davis MD, McCarthy JT, Pittelkow MR. Calciphylaxis:
Natural history, risk factor analysis and outcome. J Am Acad Dermatol. 2007
Apr;56(4):569-79.
2. Adrogué HJ, Frazier MR, Zeluff B, Suki WN. Systemic calciphylaxis revisited. Am J
Nephrol. 1981;1(3-4):177-83.
3. Nikko AP, Buningan M, Cockerel CJ. Calciphylaxis with Histologic Changes of
Pseudoxanthoma Elasticum. Am J Dermatopathol. 1996 Aug;18(4):396-9.
4. Lewis KG, Lester BW, Pan TD, Robinson-Bostom L. Nephrogenic fibrosing
dermopathy and calciphylaxis with pseudoxanthoma elasticum-like changes. J Cutan
Pathol. 2006 Oct;33(10):695-700.