Dr. William Heberden, a London physician, described the first cases of Henoch- Schönlein purpura (HSP) in In describing HSP, Heberden wrote of a. Henoch-Schönlein purpura (HSP) affects the blood vessels and causes a spotty rash. It’s not usually serious, but can sometimes lead to kidney problems. Henoch–Schönlein purpura (HSP) is a disease of the skin, mucous membranes, and sometimes other organs that most commonly affects children. In the skin.

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The young boy suffered all four disease hallmarks of HSP: They recognized that the disorder often followed upper respiratory tract infections and was not always self-limited, sometimes progressing to serious kidney involvement.

The mean age of patients with HSP is 5. In two-thirds of the cases, the disease follows an upper respiratory tract infection, with onset an average of ten days after the start of respiratory symptoms. Despite this association, no single microorganism or environmental exposure has been confirmed as an important cause of HSP.

Henoch-Schönlein Purpura • Johns Hopkins Vasculitis Center

Purpura not due to a low platelet count, caused by inflammation in blood vessels of the skin, is the hallmark of HSP. The tetrad of purpura, arthritis, kidney inflammation, and abdominal pain is often observed.

However, all four elements of this tetrad are not required for diagnosis. The microscopic hallmark of HSP is the deposition of IgA an antibody found in blood, saliva, tears, etc. The disease usually resolves within a few weeks. However, adult cases are sometimes more difficult. Skin manifestations are more variable in adults, and sometimes symptoms in adults endure longer [Figure 1, 2].

Vesiculobullous lesions These are also more common with the adult form of HSP. Adults are more prone to permanent kidney damage. HSP can be mimicked by other forms of systemic vasculitis that are more often life-threatening.

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Granulomatosis with polyangiitis and microscopic polyangiitis can also present with purpura, arthritis, and renal inflammation. These disorders both have the potential for serious involvement of other organs for example, the lungs, eyes, and peripheral nerves and carry more dire renal prognoses.

Therefore, it is very important to distinguish the difference by performing a careful evaluation including bloodwork, urinalysis, chest imaging, and possibly biopsies. HSP may be misdiagnosed as another form of vasculitis — most commonly hypersensitivity vasculitis — because of the frequent failure to perform direct immunofluorescence DIF testing on skin biopsy and the consequent failure to detect IgA.

NSAIDs may alleviate arthralgias but can aggravate gastrointestinal symptoms, and should be avoided in any patient with renal disease. Although steroids have not been evaluated rigorously in HSP, they appear to ease joint and gastrointestinal symptoms, in many but not all patients.

Steroids, however, do not appear to improve the rash; although usually, over weeks to months, the recurrent bouts of purpura usually resolve on their own.

Supportive care may involve a short course of prednisone or an NSAID, such as naprosyn or ibuprofen, if the kidneys are not involved. Keeping the legs elevated may help prevent purpura during flares of active disease. Often, the recurring purpura is less prevalent [Figure 3], and additional HSP symptoms are often absent. In many fewer cases, primarily in adults, HSP can progress from hematuria blood in the urine to renal insufficiency decreased kidney function.

HSP patients who experience this symptom should be followed more closely, with regular testing of their urine for blood and protein. Here they are occurring in a very dense pattern with coalescing lesions. Swelling around the hand and wrist. Although arthralgias are more common in HSP, arthritis can occur as well as periarticular swelling, such as the tenosynovitis shown here.

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Palpable purpura can appear in many different patterns. Sufficient pressure, such as this, causes the rupture of inflamed blood vessels. CT of abdomen showing bowel edema. This image is of a distended large bowel. This picture shows immunofluorescence testing of a skin biopsy, IgA positive. It is more common to have a purpuric outbreak on the lower extremities. However, an outbreak can occur on the abdomen, chest, or as in the case with this woman, on the upper extremities.

Note the hive-like lesions that appear larger than the papules. The rash may also be itchy. Colonoscopy of HSP-affected bowel.

Henoch-Schonlein purpura

This image shows what the lining of the bowel henkch look like when it is inflamed and swollen, as in HSP. Therefore, treatment is not indicated in all cases, and full recovery is the rule.

HSP is more common in children than adults, but has a tendency to be more severe when it occurs in adults.

In nenoch small minority of cases, HSP can cause severe kidney or bowel disease. Usually, HSP affects a child shortly after an upper respiratory infection has resolved. Occurring in a more diffuse pattern. Swelling around the ankle and foot. All information contained within the Johns Hopkins Vasculitis Center website is intended for educational purposes only. Visitors are encouraged to consult other sources and confirm the information contained within this site.

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