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.
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?
Weird Cancer
ReplyDeleteThis 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.