Comments from Faculty and Members
Robert I. Rudolph, M.D., FACP, Clinical Professor
of Dermatology, University of Pennsylvania, Philadelphia. PA,
USA on Dec 12, 2005
Did sectioning of the specimen show extrusion of calcium, and
any calcium around vessels? This looks very peculiar, and not
like PXE to me. Are radiologic studies of his vessels normal?
Is his parathyroid hormone levels and urinary calcium and phosphate
levels normal? Any angiod streaks in his eyes?
It looks like a calcifying version of eruptive histiocytosis!
Amira Adel MD, Egypt, on Dec 13, 2005
Clinically the lesion looks like histiocytosis. Histopathological
confirmation is needed.
John Andrew Carlson MD, Professor,
Divisions of Dermatopathology and Dermatology, Albany Medical
College, Albany, NY, USA on Dec 18, 2005
Strange case. PXE certainly is a good bet, but that doesn't
give you a large clump of basophilic material in upper dermis.
The photomicrograph shows a calcinosis cutis histology. Also,
instead of yellow papules and wrinkles, he has red, coalescent
papules and plaques. In PXE, there are calcified and fragmented
fibers in the mid dermis, not clumps of aggregated material.
Could this be due to a drug or external exposure of some sort?
Lionel Bercovitch MD, Medical Director,
Pseudoxanthoma Elasticum International Inc., Clinical Professor
of Dermatology, Brown Medical School, Providence, RI, USA, on
December 20, 2005
The distribution of the lesions and the histology do not suggest
PXE. In PXE, one usually sees focal calcification of dystrophic
elastic fibres in the mid-dermis and reticular deermis. The
histology here suggests some form of calcinosis cutis. I assume
that the lesions are not osteomas-the histology is not clear
at screen resolution. The chin lesions and perhaps some of the
abdominal lesions appear to perforating clinically. There is
nothing in the histology shown to support a diagnosis of histiocytosis-has
this been excluded histologically? Are other biopsies planned?
Were calcium, phosphorus, and PTH levels drawn?
Childhood PXE, while rare, tends to present with subtle yellowish
papules on the neck. Facial involvement would be extremely rare,
particularly to this degree.
Joel Bamford MD, Duluth, MN, USA on December
Interesting case, it would be nice to see follow-up note: course
and other tests ~ 3 months. Thanks for presenting this patient.
Additional comments and images from Khalifa Sharquie
MD on December 25, 2005
We know the diagnosis could be labeled as calcinosis cutis.
But what is the underlying cause? It is not clinically childhood
dermatomyositis or histologically histiocytosis X. There are
no metabolic abnormalities like hypercalcemia and the child
is healthy apart from the rash. The rash on the side of the
neck is suggestive of early PXE (attached). So by exclusion,we
put the possibility of PXE. All sections of biopsy showed nothing
apart from localised calcium deposits. Otherwise there are no
inflammatory changes to suggest any inflammatory disease like
histiocytosis X. Further evaluation will be carried out for
the benefit of the patient and doctors. Please give a reference
for any important suggestions for documentation.
Haitham Al-Qari MD, Department of Dermatology, Mount
Sinai School of Medicine, New York, NY, USA on December
Great case but doesn't suggest PXE clinically or eruptive histiocytosis
histopathologically, and the histopath picture is not that clear.
Clinically this picture is suggestive of Darier-White disease.
Suggestions: repeat the histology with specific staining.