What condition is characterized by erythroderma, lymphadenopathy, hepatosplenomegaly, and atypical T cells?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

The condition characterized by erythroderma, lymphadenopathy, hepatosplenomegaly, and atypical T cells is indeed Sezary syndrome. This condition is a leukemic form of cutaneous T-cell lymphoma and is associated with a significant proliferation of atypical T-helper cells that circulate in the blood.

Sezary syndrome is characterized by widespread red, scaly skin (erythroderma), generalized lymphadenopathy, and systemic symptoms related to the spread of neoplastic T cells, which can infiltrate lymphoid tissues, including the liver and spleen, leading to hepatosplenomegaly. Atypical T cells identified in peripheral blood are a hallmark of the disease, distinguishing it from other dermatological conditions.

In contrast, psoriasis primarily features erythematous plaques with silver scales, and while it can be associated with some degree of systemic involvement, it does not typically result in lymphadenopathy or hepatosplenomegaly. Mycosis fungoides, while also a type of cutaneous T-cell lymphoma, generally presents with skin lesions but does not usually manifest as a leukemic process like Sezary syndrome. Atopic dermatitis is primarily characterized by itchy skin and a chronic relapsing course

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