Leukemia skin are a common name for focal and diffuse lesions of the skin, subcutaneous tissue, mucous membranes of the genitals and oral cavity that occur in places of malignant proliferation of cells of hematopoietic organs and reticular tissue. They are extra-osseous foci of hematopoiesis that develop in the dermis during the transition of leukemia to the terminal stage. The foci have the appearance of erythema, erythroderma, towering papules, vesicles or bulls of light pink or dark red color, covered with smooth shiny skin, rarely crusts. In the presence of a detailed clinical picture of leukemia, the diagnosis of leukemia skin does not cause difficulties. Radiation and PUVA therapy are used for treatment.
ICD 10
C95 Leukemia of unspecified cell type
General information
In the scientific literature, several synonyms are used to designate leukemia skin at once: specific hemoderma, hematoderma, hematodermatosis, reticulosis, reticulohemoblastosis. Skin manifestations are found depending on the type of leukemia in 3-50% of patients. With monocytic leukemia, the lesion of the dermis, hypoderma is observed in every second patient, with chronic lymphocytic leukemia – in every fourth. Leukemia skin are more often diagnosed in men. In patients of both sexes older than 50 years, the probability of formation of foci of extramedullary hematopoiesis is higher than in young people. In children, less often in adults, skin infiltrates may appear at the very beginning of the disease, forming one of the so-called “debut masks” of acute leukemia.
Causes
The basis of the development of blood cancer is the malignant degeneration of hematopoietic cells of the bone marrow. Their active division leads to the formation of a clone of cancer cells, which in a short time displace normal elements from the bone marrow and begin to metastasize. Metastases of blasts and granulocytes in the dermis give rise to leukemia skin. The appearance of metastatic foci in the lungs, heart, intestines, brain leads to infiltration and disruption of these organs. For some forms of leukemia skin, for example, for primary ulcers, causal factors of occurrence have been established: previous trauma, skin irritation with chemicals, rubbing parts of clothing. But most forms of rash appear on unchanged skin.
Pathogenesis
Cancer cells enter the dermis and subcutaneous tissue with the flow of blood and lymph. Once fixed in the tissues, the cells of the metastatic focus begin to actively divide, forming large infiltrates, single or multiple elements of the rash. Each of the foci of proliferation of a clone of leukemic cells forms one element. The growth rate of the elements is largely determined by the type of cancer cells and the rate of progression of the underlying disease. As a rule, skin infiltrates are formed by blasts characteristic of this type of myeloid leukemia and have lost tissue specificity. Less common are formations consisting of mature granulocytes: promyelocytes and myelocytes up to segmentonuclear. Mature cell leukemia skin grow relatively slowly until they transform into blast leukemia skin.
Clusters of blasts in the dermis are actively disseminated with the formation of new foci on the surface of the body and in internal organs. In parallel with the dissemination of blasts, there is a process of circulatory disorders in the tissues. This is facilitated by thrombocytopenia and coagulopathy characteristic of blood diseases. Thrombocytopenia leads to subcutaneous hemorrhages, petechiae and ecchymoses. Coagulopathy is the cause of blood clots that block the blood flow in vessels of different calibers. As a result of local circulatory disorders, leaf-like crusts form on the surface of papules, foci of leukemic infiltration ulcerate.
Symptoms
The rash appears suddenly against the background of a previous malaise. In one patient, the rash may be formed by elements of different sizes and types. New elements appear within a few days. All this time, as a rule, there is a violation of the general condition, signs of intoxication. In cases where leukemia skin appear at the beginning of the disease, well-being can remain satisfactory for a long time.
Neoplasms of the skin do not cause unpleasant sensations. Infiltrates on the mucous membranes are sharply painful, with their necrotic disintegration, the pain can become excruciating. The shade of the skin over the neoplasms can vary from light green and pale pink to bluish-maroon in blast leukemids. Mature cell formations are covered with unchanged skin of a normal shade. The surface of leukemia skin is smooth, devoid of hair.
Most often with leukemia there are small papules, plaques. The elements are slightly raised above the surface of the skin, which is clearly visible in side lighting, have a rounded shape, are arranged symmetrically on different parts of the body. Consistency – from soft, dough-like to dense. The attachment of the hemorrhagic component is characterized by the formation of bubbles with a sluggish tire. For a long time, the elements remain unchanged or gradually increase in size. Their spontaneous regression is possible.
Nodes can be single and multiple, isolated or drained. Often they penetrate deeply into the hypoderm. Their size varies from millet grain to walnut. The shape is hemispherical or truncated. The consistency is dense, with chronic myeloid leukemia and acute leukemia – woody. The color is bright, rich red, less often burgundy. The blocked ducts of the sebaceous glands on the surface of the formations have the appearance of whitish grains or inclusions. Individual nodes are eroded and undergo purulent-necrotic decay. Ulcers formed at the site of nodes are resistant to local treatment.
Local leukemic infiltration, primary ulcers, specific erythroderma are rare variants of hemodermatosis. Infiltrates are formed mainly on the trunk and scalp, covered with longitudinal folds, deep grooves. The usual localization of primary ulcers in myeloid leukemia is the lower extremities, inguinal region, gums. Their diameter is 6-7 cm . The edges are uneven, covered in places. The bottom is smooth, shiny, bright red, covered with blood crusts, purulent-necrotic plaque, rarely granulations. Specific erythroderma in leukemia according to the clinical picture are indistinguishable from nonspecific.
Complications
The lesion of the skin in patients with leukemia occurs against the background of reduced immunity. In this regard, the probability of bacterial, fungal or viral infection increases many times, which aggravates the general condition of the patient, complicates treatment, worsens the prognosis regarding remission of the underlying disease and survival. Sharply painful leukemic infiltration of the oral cavity disrupts the process of chewing, eating. Weight loss can reach 10 kg per month, which quickly leads the patient to exhaustion. The spread of the purulent-necrotic process on the jaw bone increases the mobility of the teeth, provokes their loosening and loss.
Diagnostics
Algorithms for conducting diagnostic procedures in patients who have initially consulted a doctor about a skin disease and with an already established diagnosis of myeloid leukemia differ significantly. In the first case, the examination is carried out by a dermatologist, in the second by a hematologist or oncologist. With a lesion of the mucous membranes, the patient can consult a dentist. The primary diagnosis of leukemia skin is complicated. This is due to the numerous forms of skin rashes characteristic of hemoderma, the similarity of almost every form with other dermatological diseases, and in some cases also insufficient sensitivity and specificity of diagnostic tests. To make an accurate diagnosis allow:
- Thorough collection of anamnesis. It involves the identification of symptoms characteristic of the onset of leukemia, factors that increase the likelihood of developing oncology, conditions of infection with syphilis, HIV infection, leprosy, and other significant data. Detailed questioning of the patient allows you to reasonably prescribe diagnostic procedures and tests, interpret them correctly.
- Blood tests. A general blood test is prescribed, in which violations can be detected, allowing to suspect or completely exclude the diagnosis of myeloid leukemia, serological examination for syphilis, HIV. If necessary, a blood test for Mycobacterium leprosy and other infections can be carried out.
- Histological examination. Tissue samples are taken from the largest leukemia skin with the least pronounced inflammatory changes in the elements themselves and the surrounding skin. In punctates from leukemia skin, clusters of blasts are detected, less often granulocytes at different stages of maturation. The method makes it possible to make a diagnosis in 50% of cases, since the changes detected by microscopy are not always specific.
- Molecular genetic research. The polymerase chain reaction (PCR) method makes it possible to register clonal rearrangement of the T-cell receptor of lymphocytes, to establish the fact of monoclonal proliferation in lesions in the early stages of the disease. The probability of false positive results is 5%: monoclonal rearrangement is detected in a number of benign dermatoses, inflammatory processes, systemic connective tissue diseases.
- Immunohistochemical study. The method is based on the use of labeled fluorescent dyes, enzymes or electron-dense particles to detect tumor cells of a certain type. Antibodies selectively bind to blasts and make them visible under microscopy.
In dermatology, in patients without an oncological history, leukemia skin are differentiated primarily with benign processes such as diffuse neurodermatitis, lymphoplasia or primary reticulosis, fungal mycosis, sarcoidosis. In second place are infectious diseases with characteristic skin manifestations: lenticular papular syphilis, lepromatous type of leprosy. In patients with myeloid leukemia, leukemia skin must be differentiated from the skin manifestations of infections and complications of chemotherapy. These are viral and bacterial rashes, side effects of medications, and other dermatological pathology that developed simultaneously with leukemia.
Treatment
The therapeutic effect of hemoderma is aimed at the treatment of the underlying disease. The withdrawal of myeloid leukemia to stable remission leads to a gradual regression of skin manifestations. If, as a result of chemotherapy, the manifestations of hematodermatosis persist, a local effect on the foci is prescribed. To do this , apply:
- Radiation therapy. Cancer cells, unlike other cells of the body, are more sensitive to any damaging effects. The method of radiation therapy is preferable to eliminate single foci of large size. The power of X-ray radiation is selected in such a way as to affect the entire depth of leukemia skin. The number of procedures is determined individually, taking into account the previous treatment, the patient’s condition, the rate of reverse development of formations.
- PUVA therapy. The method involves exposure to skin neoplasms with long-wave ultraviolet radiation, which is preceded by the reception or local application of drugs that increase the photosensitivity of tissues. PUVA therapy is used to treat multiple elements or large-area infiltrative foci.
Prognosis and prevention
The prognosis is determined by the underlying disease. The use of modern methods of treatment contributes to an increase in the life expectancy of patients with leukemia. However, this increases the likelihood of developing serious complications of the disease, for example, damage to the central nervous system. Leukemia skin that appeared in the early stages of leukemia development, with timely access to a doctor and competent diagnosis, allow you to quickly establish a diagnosis and start treatment as early as possible. The development of hemodermatosis in diagnosed blood cancer indicates the transition of myeloid leukemia to the final stage, which is an unfavorable prognostic sign.