Pathophysiology
1) low-grade cutaneous T cell lymphoma 2) metastases occur in late stages of disease to lymph nodes and visceral organs 3) Sezary’s syndrome is a subtype with cutaneous erythroderma and circulating atypical T cells
Signs and Symptoms
1) initial presentation is usually eczematous-type skin lesions for several years before diagnosis is made 2) skin lesion progresses from patches to plaques to discrete tumor 3) loss of hair and nails 4) fever 5) chills 6) hypothermia 7) peripheral edema 8) lymphadenopathy 9) pruritus 10) high output cardiac failure
Associated Conditions
IgA nephropathy
Inheritance/Epidemiology
1) onset in mid-50s 2) more common in males and blacks
Treatment
1) if correct diagnosis is made early, focal lesion radiotherapy can provide a cure 2) after lesions become more diffuse, topical glucocorticoids, psoralen with UVA, phototherapy, and chemotherapy can be used for palliation
Tips for USMLE
1) mycosis fungoides has nothing to do with fungal infections 2) if the question mentions a 59 year-old black man who presents with a 5 year history of worsening eczema-type skin lesions, recurrent and low-grade fever, and fingernails that are starting to fall off and who is being treated by a nephrologist for an “immune disease” of his kidneys, think mycosis fungoides
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