skin myelofibrosis
Introduction
Introduction to skin myelofibrosis Cutaneous myelofibrosis, also known as cutaneous extramedullary hematopoiesis (CEH), is a chronic myeloproliferative disorder characterized by clonal hyperplasia of defective pluripotent cells in the bone marrow. Excessive production and early death of atypical megakaryocytes in the bone marrow produce a large number of platelet-derived growth factors, a potent stimulating factor for fibroblast proliferation and collagen fiber production. Extramedullary hematopoiesis is a hallmark of myelin fibrosis. Embryonic cells and committed stem cells escape large amounts from the bone marrow, enter the blood circulation, and form the same atypical cloned tumors in other organs, particularly the spleen, liver, and lymph nodes. basic knowledge The proportion of illness: 0.0025% Susceptible people: no special people Mode of infection: non-infectious complication:
Cause
Cause of skin myelofibrosis
(1) Causes of the disease
The cause is not yet clear.
(two) pathogenesis
The pathogenesis is still unclear.
Prevention
Skin myelofibrosis prevention
There is no special preventive measures for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Skin myelofibrosis complications Complication
Currently there are no related content description. Neonatal hemorrhagic purpura and blueberry muffins syndrome (blueberrymuffinsyndrome). Hemorrhagic purpura is characterized by a small reddish rash in the palm of the newborn without special clinical disease. The rash is a residual damage to the growth of physiological dermal red blood cells during embryonic development. The slightly elevated purplish red or dark blue papules that appear during the postnatal period of the newborn in the purple-black berry muffin syndrome usually resolve within 4 to 6 weeks. Adult skin lesions are scattered asymptomatic maroon or purple hemorrhagic papules, nodules or small plaques. Most often occurs in the abdomen of older men. Often the liver and spleen and lymph nodes swell, which is caused by extramedullary hematopoiesis in these organs.
Symptom
Symptoms of myelofibrosis in the skin Common symptoms Hemorrhagic papules, lymph nodes, myeloid fibrosis
Neonatal hemorrhagic purpura and blueberry muffin syndrome, hemorrhagic purpura is characterized by a small reddish rash in the palm of the newborn, without special clinical disease, the rash is the embryonic development Residual damage to rational dermal red blood cell growth, a slightly elevated purplish red or dark blue papule that occurs during the birth of a newborn in the purple-black berry muffin syndrome, usually resolves within 4 to 6 weeks.
Adult skin lesions are asymptomatic brownish or purple hemorrhagic papules, nodules or small plaques, most commonly found in the abdomen of older men, often with swelling of the liver, spleen and lymph nodes, due to the presence in these organs Caused by extramedullary hematopoiesis.
Examine
Examination of skin myelofibrosis
Histopathology: invasive or diffuse infiltration around the patchy blood vessels and around the appendage, located in the loose mucus-like matrix, a large number of blood vessels are visible in the reticular layer of the dermis, infiltrating by erythropoiesis, myeloid cells and megakaryocytes, or erythropoiesis It is composed of myeloid cells or myeloid cells. Under high magnification, clustered nucleated red blood cells and dark and condensed spherical nuclei and bright red eosinophilic vitreous cytoplasm are seen. Leder staining can confirm myeloid cells at different maturity stages. A megakaryocyte that can be calibrated with an eighth related factor antigen is characterized by a large but not typical single or multiple nucleus and a rich eosinophilic cytosol.
Diagnosis
Diagnosis and differentiation of skin myelofibrosis
According to the clinical manifestations, the characteristics of skin lesions and histopathological features can be diagnosed.
