malignant atrophic papulosis
Introduction
Introduction to malignant atrophic papulosis The disease is also known as Dego disease, Kohlmeier-Degos syndrome, lethal cutaneous and gastrointestinal arteriolarthrombosis. The disease is a disease of small arterial endometritis in the skin-intestine or other organs and then thrombosis. The cause is still unknown. It is thought to be related to autosomal dominant inheritance, autoimmune abnormality and decreased fibrinolytic activity and lentivirus infection. About 1/3 of cases have only skin damage. About 20% of cases involve the central nervous system, and rare cases can affect the eyes, heart, kidney and bladder. IgA can be significantly elevated. basic knowledge The proportion of illness: 0.004% Susceptible people: more common in young men Mode of infection: non-infectious Complications: blood in the stool, peritonitis
Cause
Causes of malignant atrophic papulosis
Causes:
The cause of this disease is unknown, and it is believed to be related to autosomal dominant inheritance, autoimmune abnormality and decreased fibrinolytic activity and lentivirus infection.
Pathogenesis:
The pathogenesis is still unclear. Some scholars believe that it is related to autosomal dominant inheritance, autoimmune abnormality and decreased fibrinolytic activity and lentivirus infection.
Histopathology:
The main lesions are small arterial endometritis in the subdermal and subcutaneous adipose tissue, swelling and hyperplasia of endothelial cells, thickening of the wall due to deposition of PAS-positive substances, thrombosis, resulting in ischemic infarction, wedge-shaped ulcer, middle membrane The outer membrane is generally unaffected, the inner elastic membrane is normal, the collagen fibers in the ischemic area are swollen, degeneration or progressive necrosis, and finally completely fibrotic and atrophic. There may be mucin deposition in the early lesions, and only in the surrounding tissues in the late stage. It can be seen that the small intestine is distributed under the infarct ulcer, white scar or perforation, and its histopathological changes are the same as skin damage.
Prevention
Malignant atrophic papulosis prevention
Strengthen nursing and nutrition to improve the patient's resistance and immunity, prevent infection should be isolated, minimize contact with pathogens, avoid close relatives, conduct premarital examination, and fertility counseling.
Complication
Malignant atrophic papulosis Complications, blood in the stool, peritonitis
Abdominal colic and blood in the stool after intestinal damage, and finally due to the development of multiple intestinal perforation and peritonitis, ocular damage to the conjunctiva may have white avascular plaque, choroid and retinal ischemia, diplopia, optic disc edema and optic atrophy .
Symptom
Malignant atrophic papulosis symptoms common symptoms scaly indigestion papule constipation diarrhea
The youngest male has the highest incidence, usually involving the skin and intestines, while the skin is often first. About 1/3 of the cases have only skin damage. About 20% of cases involve the central nervous system. Uncommon eyes can still affect the eyes, heart and kidney. And the bladder, etc., the skin damage is mainly distributed in the trunk and limbs, especially in the proximal end of the back and limbs, but the face and hands and feet are less, the primary damage is a hemispherical edematous red papule with a diameter of 2 ~ 15mm, and some damage persists during the course of the disease. Existence or absorption disappears, leaving little white skin atrophy, and most of the central necrosis, ulceration, leaving white skin with atrophy, with gray-white scales, narrow high-edge edges, or with expanded capillaries Blood vessels, damage appear in batches, as few as a few, more than a hundred, scattered distribution, less mutual integration, generally no symptoms, can last for months or years, 3 weeks to 10 years after the appearance of skin damage Intestinal damage occurs mostly in the small intestine, large intestine, mesentery, and the stomach is also involved. It is a history of insidious attacks. In the early stage, only indigestion, diarrhea or constipation may occur, and abdominal cramps may occur. Blood, and finally due to the development of multiple intestinal perforation and peritonitis, so the prognosis is poor, laparotomy, can be found on the intestinal wall multiple ovoid needle tip to a diameter of 2cm under the serosal white atrophic scar, patients without intestinal damage Among them, 33% to 50% do not have serious consequences. The eye damage is in the eyelids. The conjunctiva may have white avascular plaques, choroidal and retinal ischemic degeneration, diplopia, optic disc edema and optic atrophy.
Examine
Examination of malignant atrophic papulosis
Immunoglobulins are markedly elevated by IgA, with elevated fibrinogen levels and positive platelet aggregation tests. In addition, there is generally no positive finding.
Tissue biopsy, the main lesions are small arterial endometritis in the lower dermis and subcutaneous adipose tissue.
Diagnosis
Diagnosis and differentiation of malignant atrophic papulosis
According to clinical manifestations and histopathological examination can be confirmed.
Sometimes it needs to be differentiated from lymphoma-like papulosis, acute acne-like lichen-like pityriasis and allergic skin vasculitis.
