skin lymphogranulomatosis
Introduction
Introduction to cutaneous lymphogranuloma Most of the reported skin Hodgkin's disease is actually type A lymphomatoid papulosis, Hodgkin lymphoma (Hodgkinlymphoma), Hodgkin granuloma (Hodgkingranuloma), Hodgkin paragranomyma (Hodgkinparagranuloma), Hodgkin sarcoma (Hodgkinsarcoma), malignant lymphogranulomatosis (lymphogranulomatosismaligna). The disease is a kind of ML, with special tumor giant cells (Reed-Sternberg cells, referred to as RS cells), often with varying degrees of inflammatory cell infiltration. There is considerable overlap in these two diseases, and type A cells of lymphoma-like papulosis share a common immunophenotypic marker with Reed-Sternberg cells (RS cells). Lymphoma-like papulosis can be seen in patients with Hodgkin's disease. Primary skin Hodgkin's disease without lymph node involvement is difficult to confirm and is very rare. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: fracture jaundice hemiplegia uremia herpes zoster
Cause
Causes of cutaneous lymphogranuloma
(1) Causes of the disease
The cause of this disease is still unclear, and there are some views as follows:
1. The incidence of viral infection in close contact with patients is high. Lerine et al. proposed that the serum EB virus antibody titer in patients is increased, which is considered to be related to EB virus infection, but there is no definite evidence:
2. The incidence of radiation affecting residents of the atomic bombing area is four times higher than that of other residents;
3. May be associated with other types of ML, multiple idiopathic hemorrhagic sarcoma or leukemia;
4. Cellular immunodeficiency According to the decrease of T lymphocytes in the blood of patients, the cellular immunity is low, the normal delayed type hypersensitivity disappears, the rejection of allografts is slow, and the early lesions often start from the T-zone of lymph nodes, which is considered to be related to T cell immune surveillance. Defects lead to abnormal tumor hyperplasia of "reticular cells".
(two) pathogenesis
The pathogenesis is still unclear. Because of the controversy over the origin of RS cells, it is still to be further explored. The origin of RS cells is thought to come from T lymphocytes, B lymphocytes, tissue cells or interdigitated reticular cells or dendrites. Views such as reticular cells tend to come from reticulocytes, but they have not been confirmed.
Prevention
Prevention of cutaneous lymphogranuloma
The mechanism of cutaneous lymphogranulomatosis is very complicated, and infection is only one of the external factors. At present, there is no tendency or aggravation of lymphoma to be infected. Therefore, as a relative of lymphoma patients, the risk of lymphoma is not significantly higher than the general population. Attention to prevent infection is the key.
Reducing environmental pollution, maintaining good living habits, timely treatment of certain chronic inflammatory diseases of the body, and improving the immune function of the body may not only reduce the incidence of lymphoma, but also reduce the incidence of other malignant tumors.
Complication
Complications of cutaneous lymphogranulomatosis Complications, jaundice, hemiplegia, uremia, herpes zoster
Involvement of bones can cause fractures, exophthalmos, spinal cord compression and paralysis, central nervous system involvement can cause hemiplegia, involving the liver can cause jaundice and hepatic coma, urogenital involvement can cause uremia, patients with low cellular immunity, easy Infected with viruses, bacteria and Candida, due to low cellular immune function, at least about 15% of patients develop herpes zoster.
Symptom
Symptoms of cutaneous lymphogranulomatosis Common symptoms Nodular exfoliative dermatitis Spinal cord granuloma jaundice papules Breathing difficulties Indigestion itching coma
Patients often complain of superficial lymph nodes or lymph nodes, especially in the neck, painless progressive swelling, fatigue, loss of appetite, weight loss, irregular low fever, night sweats and itching, late involvement of any organ, due to neck and ankle Lymph nodes, can compress the arm nerve plexus and cause cough, difficulty breathing, superior vena cava syndrome, speech difficulties and other symptoms, spleen involvement can cause abdominal discomfort, indigestion, nausea, vomiting and symptoms caused by hypersplenism, involving Skeleton can cause fractures, exophthalmos, spinal cord compression and paralysis, central nervous system involvement can cause hemiplegia, peripheral neuropathy and pain, gastrointestinal involvement can cause ulcers, pain, bleeding, obstruction and malabsorption syndrome, involving the liver Can cause jaundice and hepatic coma, urogenital system involvement can cause uremia, patients with low cellular immunity, easy to cause viruses, bacteria and Candida infection.
Skin damage can be divided into specific and non-specific.
Specific damage
According to statistics from Benninghoff et al, found in 5% to 10% of patients, the primary is rare, mostly from blood source dissemination, common in patients with advanced lymph node lesions; if the corresponding lymph nodes are retrograde, can be seen in the early stage, primary The damage can occur before other symptoms, including lymphadenopathy in a few months or 5 to 10 years, the prognosis is better, the patient can survive for 10 to 20 years, and patients with secondary damage often die within a few months, damage Occurs in the scalp, the upper part of the neck and trunk, can be expressed as papules, nodules, plaques, erythroderma or eczema, even or subcutaneous nodules, ulcers can occur on the basis of nodules or plaques, Or caused by lesions such as lymph nodes or bones.
2. Non-specific damage
More common, often manifested as epidermal erosion, erythema, mossy changes, pruritus or erythroderma, etc., other such as pale skin, urticaria, polymorphous erythema, nodular erythema, red pityriasis, pemphigus, scales Disease, dermatomyositis, skin heterochromia and other damage can also occur, and sometimes can also express cyanosis, bullae, lymphedema, alopecia, palmar (sputum) keratosis and phlebitis and pigmentation, due to cellular immune function Low, at least about 15% of patients develop herpes zoster.
Examine
Examination of cutaneous lymphogranuloma
1. The blood leukocytes are normal or significantly increased, the neutrophils are increased, the lymphocytes are relatively decreased, the eosinophils are increased in about 1/5 patients, the monocytes are slightly increased, the anemia is often absent in the early stage, and the anemia is obvious in the late stage, mostly Myeloid, occasionally or hemolytic with positive anti-human globulin test.
2. Myeloid cells and megakaryocytes are often increased in bone marrow, and plasma cells and eosinophils may also increase.
3. Other erythrocyte sedimentation rate is slightly increased, basal metabolic rate is often increased (10% to 20%), liver function is abnormal, serum alkaline phosphatase is increased, plasma albumin is decreased, and globulin is increased.
Histopathology: The disease is characterized by complex tumor cells, accompanied by various inflammatory cells and capillary proliferation, often forming granuloma, occasionally necrosis, fibrosis can occur in the late stage, and RS cells have diagnostic value.
The typical RS cells are large in size, 15 to 45 m in diameter, clear in cell boundaries, rich in cytoplasm, translucent, lightly bi-color, with more than two nuclei or one lobular nucleus, and the nucleus is larger and larger. It is 8 ~ 20m; the chromatin in the nucleus is thick, often agglomerated in the nuclear membrane, uneven distribution, obvious gap, large nucleoli, shaped like inclusions, mostly round, eosinophilic or bicolor, around In the transparent ring without chromatin particles, the size of the nuclei of the binuclear RS cells is the same, and they are closely attached together. They are shaped like mirrors, also known as mirror cells. The nuclei of megakaryocytes or multinuclear RS cells are huge, distorted and overlapping. , lobulated or multinucleated, RS cells in the cytoplasm due to RNA synthesis disorder caused by RNA accumulation, strong Pyronine, so methyl green, Pyrinine staining, easy to find RS cells, this cytoplasm is acidic phosphatase and non-specific esterase Diffuse weak positive reaction, most of the RS cell membrane has I a antigen, cytoplasmic immunoglobulin positive, electron microscopy observation RS cells lack the rough endoplasmic reticulum of immunoglobulin, so the immunoglobulin is derived from tissue fluid. Organization of the disease It can be assigned to different classes.
Specific skin lesions are common in lymphocyte depletion or mixed type. Non-specific skin lesions only show chronic inflammation. Nodular or diffuse infiltration can be seen in the dermis, and the subcutaneous fat layer is extended. The epidermis is usually not affected. Gejin cells, but typical RS cells are only found in 50% of cases, infiltrating background containing lymphocytes, tissue cells, plasma cells, eosinophils and neutrophils, Hodgkin cells and RS cells are not Hodgkin's disease Uniquely, these cells are also found in lymphoma-like papulosis, anaplastic large cell lymphoma and other diseases.
Immunohistochemistry: Hodgkin cells and RS cells are often positive for CD30 (BerH2) and negative for CD45R (LCA); CD15 (LeuM1) is often positive but negative.
Diagnosis
Diagnosis and diagnosis of lymphogranuloma of the skin
diagnosis
Most Hodgkin's disease usually originates from lymph nodes, which extend retrogradely from the lymph nodes or lymphatic vessels to the skin or directly to the skin. The skin lesions are papules or nodules with or without ulceration. In advanced cases, skin miliary can occur. Dissemination, Hodgkin's disease patients often have non-specific skin lesions, and generalized severe pruritus may occur in other symptoms of the disease several months before or after the disease has been clearly diagnosed, secondary defects may occur due to scratching Itchy pruritus and pigmentation, other skin manifestations such as acquired ichthyosis, exfoliative dermatitis and generalized herpes zoster.
Non-specific skin lesions have no specificity in clinical or histopathological conditions, and the clinical morphology of specific skin lesions is not specific, and the diagnosis is mainly confirmed by histopathological examination.
Differential diagnosis
1. Lymphoma-like papulosis is benign, lesions are generalized recurrent papules or papular necrotic self-healing eruptions, histologically wedge-shaped, pro-epidermal infiltration and large atypical (RS-like) Cells, immunohistochemical phenotypes are usually CD30, CD15- and CD45R, and most cases express T cell antigens.
2. Mycosis fungus Hodgkin's disease has no single or mostly aggregated lymphocytes in the epidermis.
3. Anaplastic large cell (CD30) T-cell lymphoma is a large mother cell with densely packed sheets. Occasionally, RS cells, large mother cells CD30, CD15-, often show abnormal T cell phenotype.
