A 72 year old man, with Waldenstrom macroglobulinemia,
presented with pruritic skin lesions on the trunk
(erythematosquamous plaques) and the lower legs
(purple nodules). Waldenstrom macroglobulinemia can
present not only with cutaneous manifestations of the
known lymphoma, but can also present with other cutaneous
manifestations. These cutaneous manifestations
occur in about 5% of the patients, and these skin changes
may arise at the same time as the systemic lymphoma,
but can also precede or develop much later. There is usually
no clear relationship with possible progression of the
systemic lymphoma.
The cutaneous manifestations can be divided into I. specific
and II. non-specific cutaneous manifestations. The
specific ones are rare, and consist of the lymphoplasmocytic
B-cell infiltrate. The non-specific cutaneous manifestations
are versatile and more common; the most common
is the hyperviscosity syndroom because of increased
IgM levels in the blood (presenting with,amongst others,
swelling of the feet, bleeding of the gums and petechiae
of the hands and feet). Other non-specific cutaneous
manifestations are cryoglobulinemia associated (including
cold urticaria, Raynauds phenomenon and acral cyanosis),
caused bij deposition of immunoglobulin, or with
unknown etiology such as amyloid depositions, paraneoplastic
pemphigus or the Schnitzler syndrome.
In this patient, the two clinical presentations showed an
histologically similar picture of a dermal lymphoplasmacytic
B-cell infiltrate with IgM expression, confirming
the diagnosis of a specific cutaneous manifestation of his
systemic Waldenstrom macroglobulinemia.
Short term treatment with total skin irradiation was
unfortunately not effective. He was eventually treated
with topical clobetasol ointment with a reasonable response.
Original languageDutch
Pages (from-to)557-560
Number of pages4
JournalNederlands Tijdschrift voor Dermatologie en Venereologie
Issue number10
Publication statusPublished - Oct 2017

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