Mycosis Fungoides

by Carlo Raj, MD

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    00:01 Continuing our discussion of skin cancer.

    00:04 We have mycosis fungoides.

    00:07 First and foremost, please understand it has nothing to do with fungi, and I’ve mentioned that prior.

    00:11 And so therefore, what is it? It’s a T-cell lymphoma.

    00:14 A T-cell lymphoma.

    00:15 Remember, if we’re dealing with the skin, then you should be thinking about T-cells.

    00:20 If we’re thinking about the GI, then you should be thinking about your B-cells.

    00:25 So here, we have a lymphoma that is in your tissue of T-cell origin I said tissue.

    00:33 Because with mycosis fungoides, you’re not going to find a description clinically known as erythroderma.

    00:40 What I mean by that is what if this T-cell lymphoma wants to leave the tissue and then go onto circulation? If it does, then you call this condition Sézary syndrome.

    00:52 So the only difference between mycosis fungoides and Sézary is the location of your T-cell lymphoma.

    00:59 If the T-cell lymphoma is located in your tissue and you call this mycosis fungoides, it is the most common form of cutaneous T-cell lymphoma.

    01:09 Usually diagnosed after years and years because, well, it’s rare and the fact that you would be misdiagnosed or perhaps your doctor doesn’t even suspect it.

    01:19 Or maybe even the patient just thinks that it’s eczema or some type of dermatitis.

    01:27 As far as etiology, really idiopathic.

    01:33 And morphology becomes important.

    01:35 If you take a look at the skin here upon gross examination, you will notice that it is erythematous.

    01:40 However, you would not refer to this as being erythroderma.

    01:43 If it’s Sézary syndrome, if the T-cell lymphoma actually left the tissue and went into your circulation, you call this Sézary cells and therefore, the skin, 80% of your skin, almost all of it, would appear as being erythematous.

    01:59 You call that erythroderma, which is not the description in presentation of mycosis fungoides.

    02:05 Well-demarcated, atrophic, erythematous plaque with slight scale is your description.

    02:15 Often involves double-protected skin beneath undergarments.

    02:18 And by that, we mean there is nothing to do with exposure to UV rays as being a risk factor.

    02:25 More advanced cases may then present as your spreading or we can call it a leukemic phase.

    02:32 What does that mean? That means that now you have your T cells, your neoplastic cells that have left the tissue, have entered circulation and you call this Sézary phase and this is what I was referring to as being your erythroderma.

    02:46 Here’s the skin and what you’ll notice here is going to be your erythematous type of plaque that you can expect with mycosis fungoides.

    02:55 Where would you then expect these neoplastic cells to be located? In your tissue.

    03:02 Pathology: Atypical lymphocytes are epidermotropic or home to the epidermis.

    03:10 Pautrier abscesses formed of aggregate of atypical lymphocytes, quite significant and specific for mycosis fungoides.

    03:20 And studies of gene rearrangement often demonstrate a clonal type of population with mycosis fungoides.

    03:27 Pautrier microabscesses, it’s the aggregates of these atypical lymphocytes because you call this one of the most common cutaneous T-cell lymphoma.

    03:41 Management: Well, advanced cases at this point refer to specialist.

    03:43 advanced cases at this point, refer to specialist.

    03:46 That is as far as I’m going to go here and probably all that you need to know at this juncture in your medical education.

    About the Lecture

    The lecture Mycosis Fungoides by Carlo Raj, MD is from the course Neoplastic Skin Diseases.

    Included Quiz Questions

    1. Skin
    2. Blood
    3. Lymph nodes
    4. Gastrointestinal system
    5. Spleen
    1. Sezary syndrome
    2. Acute lymphocytic leukemia
    3. Acute myelogenous leukemia
    4. Psoriasis
    5. Mycosis fungoides

    Author of lecture Mycosis Fungoides

     Carlo Raj, MD

    Carlo Raj, MD

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