Department of Dermatology, Ruby General Hospital, Kolkata, India
Indian J Dermatol 2007:52(1):5-20
Abstract:
Mycosis fungoides (MF) is the most common group of cutaneous T-cell lymphomas. It is a rare non-Hodgkin's lymphoma of mature, skin-homing, clonal, malignant T lymphocytes, usually observed in mid to late adulthood, that initially presents in the skin as patches, plaques, tumors, or generalized erythema (erythroderma) and can involve the lymph nodes and peripheral blood. Much progress has been made in recent years in understanding the origin of the malignant T cell in MF and the patho-physiology and immunology of the disease. This recent work has made a great impact on diagnosis, prognostication, and treatment. In this review, we survey the MF literature of the last decade and highlight the major trends.
Keywords: Cutaneous lymphoma, mycosis fungoides, Sezary syndrome, T lymphocytes
Keywords: Cutaneous lymphoma, mycosis fungoides, Sezary syndrome, T lymphocytes
Summary:
1. Cutaneous T-cell lymphomas (CTCL) accounts for 2/3 of cases of primary cutaneous lymphoma.
2. MF is the most common skin lymphomas, afflicting > 50% of patients with CTCL.
3. MF is a great imitator and clinical diagnosis, particularly in the early stages, is extremely difficult.
4. The clinical presentation of MF may be divided into classic, consistent, and atypical lesions.
5. Clinical subtypes:
a. Hypopigmented MF.
b. Pagetoid reticulosis (Woringer-Kolopp disease).
c. Granulomatous slacj skin syndrome.
d. Granulomatous MF.
e. Folliculotropic MF.
6. Initial assessment: Skin biopsy for HPE/Immunophenotyping/TCR gene analysis, CT scan staging, blood tests (PBF, LFT, RP, CD4/CD8 ratios, HTLV-1 serology, TCR gene analysia), BMA and trephine biopsies. Others: PET scan.
7. Treatment options:
a. Topical corticosteroids.
b. Topical cytotoxic agents.
c. Topical bexarotenes gel.
d. Topical imiquimod.
e. Topical tacrolimus.
f. Phototherapy.
g. Photo dynamic therapy.
h. Radiotherapy.
i. Immunotherapy.
j. Chemotherapy.
k. Monoclonal antibody therapy.
l. Novel retinoids.
m. extracorporeal photochemotherapy.
n. Experimental therapy.
1. Cutaneous T-cell lymphomas (CTCL) accounts for 2/3 of cases of primary cutaneous lymphoma.
2. MF is the most common skin lymphomas, afflicting > 50% of patients with CTCL.
3. MF is a great imitator and clinical diagnosis, particularly in the early stages, is extremely difficult.
4. The clinical presentation of MF may be divided into classic, consistent, and atypical lesions.
5. Clinical subtypes:
a. Hypopigmented MF.
b. Pagetoid reticulosis (Woringer-Kolopp disease).
c. Granulomatous slacj skin syndrome.
d. Granulomatous MF.
e. Folliculotropic MF.
6. Initial assessment: Skin biopsy for HPE/Immunophenotyping/TCR gene analysis, CT scan staging, blood tests (PBF, LFT, RP, CD4/CD8 ratios, HTLV-1 serology, TCR gene analysia), BMA and trephine biopsies. Others: PET scan.
7. Treatment options:
a. Topical corticosteroids.
b. Topical cytotoxic agents.
c. Topical bexarotenes gel.
d. Topical imiquimod.
e. Topical tacrolimus.
f. Phototherapy.
g. Photo dynamic therapy.
h. Radiotherapy.
i. Immunotherapy.
j. Chemotherapy.
k. Monoclonal antibody therapy.
l. Novel retinoids.
m. extracorporeal photochemotherapy.
n. Experimental therapy.
Source: IJD
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