A 50-year-old female presented with ulcerated firm to hard nodules over the covered pas of her body, along with generalized erythema, edema and extreme pruritis. Initially they were barely palpable erythematous, well defined patches, which later on conveed to plaques and became itchy and ultimately turned into the current lesions. Cervical lymphadenopathy was also noted. Skin biopsy from the lesion was taken. Peripheral blood smear also showed abnormal cells. Which is the most likely diagnosis: –
Correct Answer: Sezary disease
Description: This is a case of sezary syndrome. 1st image shows the classical Sezary cell with cerebriform nucleus. 2nd image shows intraepidermal collection of atypical lymphocytes (Pautrier's microabscesses) a feature of epidermotropism and a band of lymphoid cells, some atypical, in the upper dermis. Diagnosis is based on: 1. Chronicity of lesions and poor response to conventional therapy. 2. Lesions predominantly on covered pas. 3. Characteristic stages: Well-defined patches of bizarre shapes with cigarette paper atrophy indurated itchy plaques Nodules which often ulcerate 4. Typical histology: Intraepidermal collection of atypical lymphocytes (Pautrier's microabscesses) a feature of epidermotropism 5. A band of lymphoid cells, some atypical, in the upper dermis.
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