Mycosis fungoides

Mycosis fungoides (MF) is the most prevalent type of primary cutaneous T-cell lymphoma,
representing 50% of all primary cutaneous lymphomas and 60-70% of all cutaneous T-cell
lymphomas (CTCL). It is characterized by a progression from patches to plaques to tumors, with a
histological hallmark of an epidermotropic infiltrate of small to medium-sized CD4+ T-cells.
MF is T helper T cell Lymphoma MARKER CD3, CD4

ETIOLOGY
Etiology The etiology of MF is unknown.

TRIGGERING FACTOR :

1. Genetic Predisposition :
2. Environmental exposure :
to persistent (chronic ) antigenic stimulation
a. Industrial : worker in fin industries of chemicals, metals, and pesticides
b. Infectious agents
MOSTLY VIRUS INFECTION e.g. human T-cell lymphotropic virus (HTLV) I/II,
human immunodeficiency virus (HIV), Epstein-Barr virus (EBV), cytomegalovirus
(CMV), and human simplex virus (HSV).

3. Immunological factors .
i. In the early stages of MF (patch, plaque), T cells show a cytokine profile of Th1
(IFN-y, IL2, IL12)
ii. In advanced stages (tumor), a shift in cytokine profile from Th1 to Th2 with the
secretion of cytokines IL4, ILS, IL10

Epidemiology and Risk Factors:

MF typically affects individuals in late adulthood with a median age of onset around 55–60 years
and exhibits a male predominance, particularly in white patients.
However, diagnosis in patients under 30 years of age is more common among Asians, Pacific
Islanders, and Black Americans.
Clinical Presentation:
MF manifests as chronic itchiness, often preceding visible signs of disease.
It progresses slowly from patches to plaques and eventually tumors.
THERE ARE CLASSICAL AND NON -CLASSICAL MF presentation

CLASSICAL MF PRESENTATION :

The patch stage

Characterized By Poorly defined, irregular, finely scaling, red patches of variable size often with
atrophic (thin, wrinkled) skin
Located primarily in sun-protected areas.
Often asymptomatic

THE Plaques stage

Well-demarcated annular or arciform itchy thickened lesions
Colour often red, violaceous, or brown, sometimes scaly
plaques can become quite large with central regression
Develop from patches or de novo
Initially involves less than 10% of the body surface area (BSA), becoming widespread later

THE tumors STAGE

are large irregular lumps.
color: Deep red to violaceous,surface often shiny

NON CLASSICAL MF PRESENTATION

variants
● Syringotropic MF
● Bullous MF
● Solitary MF
● Mycosis fungoides palmaris et plantaris
Forms resembling (amongst others):
○ Pigmented purpuric eruption (capillaritis)
○ Granuloma annulare
○ Acanthosis nigricans
○ Pyoderma gangrenosum
○ Vitiligo
○ Ichthyosis
○ Psoriasis

Skin staging of mycosis fungoides

1. T1, limited (<10% BSA) patches/plaques
2. T2, generalised (>10% BSA) patches/plaques
3. T3, tumours
4. T4, erythroderma.

**Diagnosis and Differential Diagnosis:**

Diagnosis requires careful clinicopathological correlation.
Dermoscopy aids in distinguishing MF from other dermatoses.
Multiple skin biopsies necessary, with histopathology revealing malignant CD4+ T-cells.

Differential diagnoses include dermatitis, psoriasis, other inflammatory dermatoses AND

malignancies like other cutaneous lymphomas.

**Complications and Prognosis:**

❖ Complications include
1. ulceration,
2. extracutaneous spread
3. transformation to large cell lymphoma
4. .Erythrodermic mycosis fungoides — unlike Sézary syndrome there are no/few
circulating ‘Sézary cells’ in the peripheral blood

❖ The prognosis is relatively favorable, with a 5-year survival rate of 87%.

 ❖ individual lesions can regress spontaneously.
❖ Survival predictors include age, skin stage, and the presence of extracutaneous disease.
❖ Hypopigmented and poikilodermatous variants have better outcomes compared to classic MF,
❖ while folliculotropic and granulomatous MF are more aggressive.

Treatment:
Treatment aims to manage symptoms as MF is not curable.

Topical treatments
steroids and chemotherapy are common for early-stage disease.
● Topical steroids
● Topical chemotherapy eg, nitrogen mustard, carmustine
● Topical bexarotene( class of retinol)

Phototherapy, radiotherapy, and systemic treatments like chemotherapy and immunotherapy are
options for more advanced cases.

Procedural treatment
​ Phototherapy — PUVA, narrowband UVB
​ Radiotherapy and whole-body total skin electron beam therapy
​ Extracorporeal photopheresis

Systemic treatment
​ Chemotherapy — mycosis fungoides is relatively chemoresistant
​ Immunotherapy — interferon-alpha
​ Oral retinoids and rexinoids (eg, bexarotene)
​ Other therapies — including denileukin diftitox, monoclonal antibodies, histone deacetylase
inhibitors

Only brentuximab vedotin (for CD30+ MF) and allogeneic haematopoietic are the only stem cell
transplant alter the natural history of the disease.
Conclusion:
Mycosis fungoides diagnostic challenges BUT can be managed effectively Based on
disease stage and patient preferences.
Early detection and appropriate treatment are crucial for improving outcomes and quality of life
for patients.
characterized by a specific skin lesion in the form of universal erythroderma.
The average age of onset of the disease is 58-60 years.
It occurs in one third of patients.

Depending on the clinical characteristics and the course of the pathological skin
process divided to : , dry and exudative erythroderma are distinguished.

Dry erythroderma
is characterized by
● slow dynamics of the tumor process,
● absence of weeping
● fine-plate peeling
● less pronounced skin itching.
● Hyperemia is combined with infiltration,swelling and peeling of the skin.
● With severe infiltration of the skin, some patients experience distortion of
facial features in the form of thickening and folding of the skin.
● The color of the skin is red with various shades (blue-brick or brown).
 ● the general condition remains satisfactory for a long time, and the
subcutaneous lymph nodes enlarge later.

The exudative form

is characterized by
● the rapid development of swelling, infiltration, large-plate abundant
peeling, and oozing.
● , erythroderma develops rapidly spreading over the entire skin without
previous rashes.
● the general condition is early disturbed with an increase in temperature.
● They complain of headaches, insomnia, loss of appetite, itchy skin, a
constant feeling of chills and skin tightness.
● color : ashy-brown tint (melasma variant).
● Patients complain of
○ unbearable itching,
○ burning and swelling of the skin,
○ severe chills.
○ impaired sweating of the skin,
○ total alopecia,
○ onychodystrophy,
○ hyperkeratosis of the palms and soles
○ , enlarged peripheral lymph nodes
○ cachexia.
● The course is long-lasting - for many years and even decades.
● In the tumor stage, when the process spreads and involves internal organs,
the prognosis is unfavorable,
● disease can progress to high-grade large cell lymphoma, in which the life
expectancy not exceed 3 years
●
●