Hemorrhagic Vasculitis: What It Is, Symptoms, and Causes

Hemorrhagic vasculitis is a disease in which the walls of small vessels become inflamed and a rash of small hemorrhages appears on the skin. It is most often detected in children, but it also occurs in adults. Below is what is known about the causes, symptoms, and monitoring for this diagnosis.

What hemorrhagic vasculitis is and how it relates to Henoch-Schönlein purpura

This is the most common disease in the group of systemic vasculitides. It is based on aseptic inflammation of the walls of microvessels and multiple microthrombus formation, which affects the vessels of the skin and internal organs, most often the kidneys and intestines. There are many names: Schönlein-Henoch purpura, Schönlein-Henoch disease, rheumatic and allergic purpura. The term "hemorrhagic vasculitis" has been accepted in Russian-language medicine since 1959; abroad, it is called "Henoch-Schönlein purpura."

Search queries include the abbreviation "hemor vasculitis" and the distortion "hemorrhoidal vasculitis." These refer to the same inflammation of microvessels of the skin and internal organs, not to a disease of the veins of the rectum.

Causes and mechanism: is it an autoimmune disease

Immune complexes and activated components of the complement system circulate in the blood. Normally they are removed by cells of the phagocytic system; when they accumulate excessively, they are deposited on the endothelium of the microcirculatory bed, activate complement, and damage the vascular wall. Hemostasis then shifts: platelets are activated, coagulability increases, antithrombin III decreases, von Willebrand factor increases, and fibrinolysis is suppressed.

In essence, this is immune complex-mediated vascular damage—hence the frequent question of whether this disease is autoimmune. In 66–80% of patients, an upper respiratory tract infection preceded the disease; typhus, paratyphoid, measles, certain medications, food allergy, insect bites, hypothermia, and sometimes pregnancy are also mentioned.

Symptoms: rash, joints, abdomen, kidneys

There are several manifestations, and the combination differs from person to person.

• Skin: palpable purpura, with elements slightly raised above the skin. First the distal parts of the legs, then the thighs and buttocks, rarely the arms, abdomen, and back. After a few days the rash turns brown and disappears, leaving no scars.
• Joints: 59–100% of cases, more often in adults. The pain is migratory, affecting large joints of the legs, less often the elbows and wrists, usually lasting no longer than a week. In a quarter of patients, especially children, joint pain precedes the rash.
• Abdomen: in about two thirds—cramping pain, nausea, vomiting; moderate bleeding occurs in up to 50% of cases, life-threatening bleeding in no more than 5%.
• Kidneys: from 10 to 60% according to various data, more often after other signs of the disease.

The rash resembles small bruises or blood spots; there may be many of them or only a few. Glass test: press the edge of a clear glass against the skin; if the spots do not blanch—see a doctor without delay.

Hemorrhagic vasculitis in children and in adults

It occurs more often in children. A fulminant course often develops in children under 5 years of age. By course, an acute variant is distinguished (resolves within 2 months), subacute (up to 6 months), prolonged (longer), and chronic. Activity is assessed by three degrees: the first—satisfactory condition, ESR up to 20 mm/h; the second—fever above 38 and ESR 20–40 mm/h; the third—severe condition, ESR above 40 mm/h, anemia and decreased platelets are possible. The disease is not contagious: the child returns to school when they feel better.

How the disease is diagnosed

The doctor assesses the rash, the condition of the joints and abdomen, as well as ESR, leukocytes, platelets, a urine test, and blood pressure. In the abdominal form, endoscopy reveals hemorrhagic or erosive duodenitis. After an episode—regular examinations for 6–12 months with urine and blood pressure checks.

Possible complications and when to see a doctor urgently

Usually the disease ends in spontaneous remission or complete recovery 2–3 weeks after the first rash. But there can be a severe course lasting several months and a small chance of persistent kidney damage—chronic kidney disease. Complications include intestinal intussusception, perforation, and peritonitis. The disease can recur: if symptoms return, a prompt medical consultation is needed.

The rash alone is not a reason to call an ambulance. Call 103 or 112 if the spots do not blanch when pressed with glass and the person feels very unwell: it is painful to look at bright light, the neck is stiff.

You can come to an appointment with questions: how often to check urine and blood pressure, which signs mean the disease has returned, whether a nephrologist examination is needed. This material is for reference and does not replace a doctor's consultation.

This content is for reference and does not replace a doctor's consultation. Medical disclaimer