Title: GZ / The Grenz Zone Season 1 / Ep 7
Tags: GZ DermnetNZ Waikato

Q: [...] is the most common primary cutaneous lymphoma and is mediated by [...]-cells
A: Mycosis fungoides; T
Back Extra: Most cutaneous lymphomas are non-Hodgkin's lymphomas of T-cell origin, of which MF is the most common

Q: Clinical Presentation of Mycosis Fungoides
Erythematous, occasionally pruritic, oval scaly patch in sun-protected “bathing suit” distribution
Classically slow progression through 3 stages (although it may not progress or start at stage 1): [...], [...], and [...]
A: Patch; plaque; tumor
Back Extra: Patch: round or oval patches 1-5 cm in width and may be annular or polycyclic. Itchy and appear on sun-protected areas (e.g. upper thighs and buttocks) in a “bathing suit” distribution
Plaque: well-demarcated indurated scaly plaques that take on a variety of shapes with a violaceous to red-brown color
Tumor: rapidly enlarging nodules that develop within patches or within plaques of MF

Q: Mycosis Fungoides classically progresses through 3 stages (although lesions may start at plaque and/or may not progress)
Patch: round or oval patches 1-5 cm in width and may be annular or polycyclic. Itchy and appear on [...] areas in a [...] distribution
Plaque: well-demarcated indurated scaly plaques that take on a variety of shapes with a [...] color
Tumor: rapidly enlarging nodules that develop within patches or within plaques of MF
A: sun-protected; “bathing suit”; violaceous to red-brown
Back Extra: e.g. upper thighs and buttocks
Bathing suit distribution is a nice pearl since UV therapy often helps to treat MF
Patch
Plaque
Tumor

Q: Other variants of mycosis fungoides that may not be psoriasiform include [appears as papules and plaques centered in hair follicles on head and neck region], [includes Woringer-Kolopp and Ketron-Goodman],  and [causes sagging skin in axilla and groin] MF, among others
A: folliculotropic variant; pagetoid reticulosis; granulomatous slack skin
Back Extra: Folliculotropic variant may be associated with alopecia
Woringer-Kolopp usually describes solitary psoriasiform plaques on the distal extremities lesions
Ketron-Goodman variant, which is more disseminated and has a worse prognosis
Other variants include hypopigmented, verrucous, interstitial, and poikilodermic MF
Folliculotropic on lower extremity
Pagetoid reticularis

Q: What should you think about when you see psoriasis in a sun-covered area?
[...]
A: Mycosis Fungoides

Q: A key finding in the histology of mycosis fungoides is [...], which atypical lymphocytes seen in the
epidermis with minimal spongiosis, as opposed to [...], in which atypical lymphocytes will also beseen in the
epidermis, but with more spongiosis
A: epidermotropism; exocytosis
Back Extra: Epidermotropism
Exocytosis

Q: In addition to epidermotropism and a band-like distribution of atypical lymphocytes at the DEJ
mycosis fungoides may present histologically with [...], which are atypical lymphocytes with large hypochromatic nuclei that appear in clusters
A: Pautrier’s microabscess

Q: Epidermotropism may be diminished or absent in the [...] phase of mycosis fungoides
A: tumor

Q: The ratio of CD4:CD8 [...] as Mycosis Fungoides progresses
A: increases
Back Extra: Normally 1:1 in other inflammatory diseases
<4:1 = less progression = longer survival
>10:1 worse prognosis (seen in Sézary syndrome)

Immunohistochemical staining for Mycosis fungoides is typically yields a phenotype that is
CD3 +, CD4 +, CD8 -, and CD30 -
Back Extra: Exception for hypopigmented variant of MF favoring children and darkly pigmented pt: CD4-, CD8+

Q: In hypopigmented variant of MF favoring children and darkly pigmented patients yields a phenotype that is CD4 [...], CD8 [...]
A: -; +
Back Extra: Considered a cytotoxic variant
Normally CD4 + and CD8 -

Q: Loss of T-cell markers may also be seen and used to identify mycosis fungoides
Loss of CD [...] is the most common but least specific change
Loss of CD [...] & CD [...] are less common but more specific
A: 7; 5; 2
Back Extra: Note: CD7, CD5, and CD2 are T cell markers

Q: Patch and plaque mycosis fungoides are both characterized by a(n) [...]-like distribution of atypical lymphocytes at the [...]
A: band; dermoepidermal junction
Back Extra: Patch: band-like distribution of atypical lymphocytes @ DEJ
Plaque: similar histo findings to patch stage, but more dense band-like
infiltrate in the upper dermis + more epidermotropism

Skin directed therapy for MF includes topical high-potency corticosteroids (clobetasol), nitrogen mustard, narrow band UVB, Psoralen + PUVA, or radiation therapy

Q: Refractory MF may be treated with systemic treatment, such as [...] and [...] like [...], for which a common side effect is central hypothyroidism
A: Interferon-alpha; oral retinoids; bexarotene

For rapidly progressive MF or MF that has lymph node/visceral involvement, systemic chemotherapy such as the CHOP regimen, may be used
Cyclophosphamide
Hydroxydaunorubicin (Doxorubicin)
Oncovin (Vincristine)
Prednisone