Stages and what the disease looks like
The disease usually goes through several successive stages. First, spots appear: pinkish or brownish, slightly flaky, often in areas covered by clothing - on the buttocks, thighs, abdomen, chest. They can persist for years, fading and then reappearing. Then plaques form - compacted, raised areas above the skin with clear boundaries and more pronounced itching. In the later stages, dense nodes are formed that are prone to ulceration. A separate option is erythroderma, when almost all of the skin turns red and peels.
- Stage of spots on closed areas of the body
- Stage of compacted plaques
- Stage of nodes with ulceration
- Erythrodermic form
- Possible areas of pigment loss
- Transition between stages can take years
Why is the diagnosis made late?
In the early stages, the skin changes and microscopic appearance are almost indistinguishable from chronic eczema, psoriasis and contact dermatitis. Moreover, external hormonal agents provide temporary improvement, which confirms the impression of ordinary dermatosis for the doctor and patient. Malignant lymphocytes are few in number at this stage, and a biopsy alone is often insufficient. Therefore, for rashes that persist for more than six months, return after discontinuation of treatment and are located on closed areas of the body, the dermatologist repeats the biopsy, sometimes several times from different lesions.
- Similarities with eczema and psoriasis
- Temporary relief from external hormones
- Few tumor cells at an early stage
- A single biopsy may not be informative
- Indication for repeat biopsy: persistence of lesions
- A biopsy is taken after a break in external treatment
Diagnostics
The key study is a skin biopsy with histological and immunohistochemical studies showing the penetration of atypical T lymphocytes into the epidermis. It is complemented by molecular analysis for rearrangement of the T-cell receptor gene, confirming that the lymphocytes originate from the same clone. It is important that before the biopsy there is a break in the use of external hormonal agents, otherwise the picture will be blurred. To assess the prevalence, an examination of the entire skin is performed, calculating the area of the lesion, a general blood test with a search for atypical cells, LDH, ultrasound of the lymph nodes, and, if necessary, a CT scan.
- Skin biopsy with immunohistochemistry
- T cell receptor gene rearrangement
- Break in external treatment before biopsy
- Assessment of the area of skin damage
- General blood test with leukemia formula
- LDH and biochemical parameters
- Ultrasound of lymph nodes, CT if necessary
Treatment in the early stages
While the disease is limited to the skin, treatment is aimed at the skin itself and is relatively easily tolerated. External glucocorticoids, retinoid-based agents, as well as narrow-spectrum ultraviolet phototherapy or in combination with photosensitizers are used. For individual lesions, local radiation therapy is possible, to which the disease is very sensitive. The goal at this stage is control of rash and itching, not complete cure, and aggressive chemotherapy is not used here because it does not improve prognosis but reduces tolerability.
- External glucocorticoids
- Topical retinoids
- Ultraviolet phototherapy
- Local radiation therapy to selected lesions
- Emollients and skin care
- Chemotherapy is not indicated in the early stages
Common stages and living with the disease
When nodes appear, lymph nodes or blood are affected, systemic treatment is started: oral retinoids, interferon, targeted drugs, chemotherapy if necessary, and in some situations - irradiation of the entire skin with an electron beam. A separate important task is the fight against itching and dryness, which greatly affect the quality of life: regular use of emollients, cool showers, mild detergents, and cotton clothes help. Due to a broken skin barrier, the risk of infections is increased, so any suppuration requires timely treatment.
- Systemic retinoids and interferon
- Targeted drugs
- Chemotherapy for common forms
- Irradiation of the entire skin with an electron beam
- Daily use of emollients
- Cool showers and mild cleansers
- Timely treatment of skin infections
