A 56 year old African American male presents with severely pruritic skin rashes. For the last few years, he's had nonspecific scaling skin lesions and biopsies were unremarkable. A primary care doctor gave him the diagnosis of "nonspecific dermatitis." But now he comes to your dermatology clinic with scaly patches and plaques >5cm in diameter. They are confined to the trunk. He also has generalized erythroderma and lymphadenopathy.
A skin biopsy shows this:
The red arrow indicates a specific type of microabscess. The blue arrow shows mononuclear cells in the epidermis. A blood smear shows this:
Challenge: This is one of the weirder cancers you've seen. What is it?
Both images shown under fair use.
Friday, May 30, 2008
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Weird Cancer
This is Sezary syndrome, an aggressive leukemic variant of mycosis fungoides. Mycosis fungoides is an extranodal indolent non-Hodgkin’s T-cell lymphoma with skin involvement. Pathogenesis is unclear; epidemiology is as described in this case. The clinical presentation may involve a nonspecific premycotic period and then skin lesions (patches, plaques, tumors, erythroderma, poikiloderma). Sezary syndrome involves circulating Sezary cells, mononuclear cells with a cerebriform nucleus. There many be extracutaneous disease. The histologic image shows Pautrier microabscess (red arrow) and epidermotropism (blue arrow).
Sources: UpToDate; missinglink.ucsf.edu; healthsystem.virginia.edu.
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