In between patients one of my nurses stopped me and said that Mrs. Brown was here with her four-year-old daughter. She was panicked because of her daughter’s symptoms and couldn’t wait to make an appointment. The nurse was instructed to put her in a room and I would see her next.
Upon entering the room I recognized Kelly who had been here for a physical just one month before. Mom was on the verge of tears. As it turned out, Kelly started developing a rash on her legs a few days ago. She might have had a low-grade fever, but that was not enough of a concern to even take her temperature. The worry was about the rash because last night, when helping Kelly get ready for bed, the rash seemed to have spread to her buttocks. It was now slightly raised and almost a bruise-like color. Mom told her husband that she was going to make an appointment first thing in the morning.
When Kelly got up at the usual time this morning Mom was curious to see if the rash had subsided. She was startled to find that not only was the rash worse, but Kelly’s ankles were swollen and red. She could barely bear any weight on them. That is why the child was put in the car and driven right to the office.
The nurse had already done a blood pressure on Kelly and had taken her temperature. Both were normal. Kelly’s exam was significant for an impressive rash on her lower extremities that was most concentrated on her buttocks and the back of her thighs. The rash was a deep red color, slightly raised, and in some areas seemed to have coalesced into larger blotches. Some had a hive-like appearance and those that were older had almost a purplish, bruised appearance. Her ankles were swollen, and there was a suggestion of redness to the swollen area along with palpable warmth. It was uncomfortable for me to passively range and move her ankle joints and walking was very difficult for her. The rest of the exam was normal.
Some simple blood tests were sent to the lab. Mom was told that there was more concern about the urinalysis, which we collected in the office. It was completely normal. Kelly’s mom was then told that she had Henoch-Schonlein purpura (HSP). With a stunned look on her face I went on to explain that I was sure Kelly had HSP and the child was in no danger; she would probably be just fine. After Kelly was dressed we would talk about HSP and what it is exactly.
HSP is an interesting syndrome or constellation of symptoms that can affect any age, but it is far more common in children than adults. It is most common in children two-to-eight years of age, and it affects boys more than girls by almost two-to-one. No one knows the exact cause of HSP, but some of the agents that have been implicated include drugs, viruses or strep. It is felt that whatever the triggering agent is, it sets up an allergic reaction of a sort. This causes an inflammation of tiny blood vessels , or a vasculitis. HSP can affect different organ systems.
In the 1830’s Dr. Schonlein described a disease that caused a rash on the lower extremities and also caused swollen and tender joints. In the 1870’s Dr. Henoch described an illness that caused abdominal pain and kidney involvement. It wasn’t until later that these two disease processes were one in the same.
HSP is a process that can affect the skin giving the characteristic rash that usually involves the legs and buttocks. However, the rash can occur anywhere on the body. It is a result of a vasculitis, or inflammation of tiny blood vessels, near the surface of the skin. The rash may occur in crops.
The joint involvement of HSP is actually an arthritis, which occurs in approximately two-thirds of children with this condition.. It usually affects the larger, weight-bearing joints such as the knees and ankles. The swollen, painful and tender joints of HSP usually resolve in a matter of days without any permanent symptoms.
HSP can also affect the gastrointestinal tract that occurs in approximately 50 percent of children who are affected. There is a vasculitis that occurs in the wall of the intestine. This can cause severe, crampy abdominal pain and even bloody stools. In some instances, the swelling of the wall of the intestine from the vasculitic area can cause the intestine to twist or telescope on itself. This can cause an intestinal obstruction that requires surgery. Fortunately, this complication is not common.
The kidneys can be involved in HSP, but this may be more subtle. One may find that blood and protein are lost through the kidneys but this would only be detected by checking the urine. The hematuria and proteinuria occur in about one-third of the patients with HSP, and it may be a late manifestation. Whenever a diagnosis of HSP is made, one must follow the urines of these patients for at least two months before being sure the kidneys will not be involved.
HSP does not have to affect ALL of these organ systems for a diagnosis to be made. It may affect the skin and joints, the skin and kidneys, or the skin, intestinal tract and joints. It is variable. There is no predicting which organs will become involved or how severe the symptoms will be.
Overall, the outcome of a patient with HSP is excellent. The majority of cases are self-resolving with only symptomatic care being required. The symptoms typically resolve in a matter of days. One indication for specific intervention is in the case of severe abdominal pain or bloody stools. In these cases, one would start corticosteroids with the hope of preventing intestinal obstruction. When a diagnosis of HSP is first made, it is imperative to follow the urines closely for blood and protein. If there is kidney involvement a certain percentage can go on to have chronic kidney insufficiency, and these patients must be identified.
HSP can recur, although this is not very common. What is of interest is the fact that if it recurs, it affects the very same organ systems that were involved the first time.
Kelly’s mom was pleased with our talk about HSP and her mind was at ease. Sure enough, the child’s urine was negative. The blood work was normal as well and Kelly had a fairly mild case. She was back to her baseline self in around five days. I monitored the urine every two weeks for a total of two months, and she never did develop any kidney involvement.