Ultrasound examination of the abdomen showed multiple small
echogenic foci with distal shadowing that was seen bilaterally in
the renal parenchyma at the corticomedullary junction. This finding
was suggestive of calcification. No other significant abnormality
was detected on ultrasonography (Figure 1).
The patient had asymptomatic, skin-colored, flat-topped papules
involving the neck and periumblical areas for the last 6 months.
Examination revealed 1- to 3-mm yellowish-white papules coalescing
to form plaques distributed in the flexures, neck, and the
periumbilical area with characteristic "chicken-skin" appearance on
the neck (Figure 2). Oral mucosa, scalp, palm, and sole were not
LABORATORY AND PATHOLOGICAL RESULTS
Biochemical investigations revealed a normal hemogram; serum
calcium, potassium, uric acid, phosphorus, albumin, urea, and
creatinine levels were also normal. Urine examination revealed a
24-hour protein level of 500 mg and normal pH, and there were no
blood cells on microscopic examination. Urinary excretion of
calcium was estimated (as a calcium:creatinine ratio) and was
normal. Stool was negative for occult blood.
Electrocardiogram and 2-dimensional echocardiography of the
heart were normal (not shown). Blood pressure was within normal
limits. Skin biopsy was performed from the neck, and the specimen
was stained with Verhoeff-van Gieson stain, which revealed
clumped-up, darkly staining elastic fibers in the mid dermis.
Von-Kossa stain revealed basophilic deposits of calcium.
The patient was asked to follow up regularly. Subsequently, a
detailed examination of the family and first-degree relatives was
undertaken, which revealed biopsy-proven PXE, without any other
complication, in the sister of the patient reported here. The
patient was advised to avoid any form of head trauma to prevent
retinal hemorrhage. Follow-up after 1 year did not reveal any
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Prepared by Sumer Kumar Sethi, MD (Radiodiagnosis); Ravi
Solanki, MD (Radiodiagnosis); Pitamber Sonkar, MD (Radiodiagnosis);
Kabir Sardana MD (Dermatology); Vibhu Mendiratta MD (Dermatology),
Departments of Radiodiagnosis and Dermatology, Lady Hardinge
Medical College and Associated Kalawati Saran Childrens Hospital,
New Delhi, India.
- USG scanner (GE Healthcare, Waukesha, WI)
- LOGIQ 400 system (GE Healthcare)