Middle East Journal of Family Medicine

Maher khader MD (1), Wajdi Ammayreh MD (1), Ahmed Issa MD(1),
Salah Abdallat MD (2), Basem Momani MD (1)

Correspondence to:
Email: a_alissa79@yahoo.com, P.O. box 6251, Tel: 0777416221
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ABSTRACT

Objective: To study the epidemiological and clinical manifestation of Henöch-Schönlein purpura (HSP) in Jordan.
Setting: Princess Hia Hussein Hospital (Aqaba) and Queen Alia military hospital (Amman).
Methods: This retrospective study was carried out during the period between January 2002 and January 2006. A total number of 30 patients were studied regarding the age, gender, clinical manifestation, laboratory findings and treatment.
Results: Of the 30 patients, 17 (56.7%) were males and 13 (43.3%) were females. age ranged between 1-12 years with a mean age of 5.75 years. Seasonal distribution of admissions was highest in the winter months . Sll patients had the characteristic skin rash , 83.3 % of patients had joint manifestations and 53.3 % of patients had gastrointestinal manifestations . Treatment was supportive in 26 (87.7 %) patients, whereas short courses of steroids were started in the remaining 4 (13.3 %) patients.
Conclusion: Henoch-schönlein purpura is a self limiting disease with an excellent overall prognosis and generally treatment is unwarranted.

Keywords: Henoch- Schönlein purpura, treatment, epidemiologic, clinical manifestation.
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HSP is the most common vasculitic disease of childhood (1, 2, 3). It is an IgA-mediated, autoimmune, hypersensitivity vasculitis of small vessels (4, 5, 6). It was first reported by Heberden in 1806. The association of purpura and joint pain was described by Schönlein in 1837, who termed it peliosis rheumatica. Henöch added a description of four children with skin lesions associated with colicky abdominal pain, gastrointestinal hemorrhage and joint pain in 1874, and in 1899 pointed out that renal involvement sometimes occurred(2,7). It has an annual incidence of approximately 13.5-18 /100,000 children (1), and peaks during winter (2). Most of the cases occur between 2-8 years of age with a male-to-female ratio of 2:1(5, 8). In 1990, the American college of Rheumatology defined HSP by the presence of 2 or more of the following criteria: age of disease onset 20 years or younger; palpable purpura; acute abdominal pain (bowel angina) and granulocytic infiltration in the walls of arterioles or venules (1, 9).

The records of all children diagnosed as having HSP from January 2002 to January 2006 were reviewed. The criteria for diagnosis of HSP were based on the classical description of skin rash together with either joint involvement (arthralgia and / or arthritis) or gastrointestinal manifestations (abdominal pain and /or gastrointestinal bleeding).

Of the 30 patients, 17 (56.7%) were males and 13 (43.3%) were females. The age ranged between 1-12 years with a mean age of 5.75 years. Seasonal distribution of admissions showed that most of the cases occurred in winter . The frequency of different signs and symptoms is shown in table I. All patients had the characteristic skin rash, 25 patients had joint manifestations, including arthritis in 13 patients, involving the larger joints particularly the knees and ankles. 16 patients had gastrointestinal manifestations in the form of abdominal pain and /or gastrointestinal bleeding, 7 patients had renal involvement in the form of hematuria, proteinuria or both. Renal function tests and blood pressure were normal in all patients on admission and a short follow-up, one patient had scrotal edema and another one developed multiple itracerebral hematomas and unfortunately died.
The laboratory findings are shown in table II. Throat swabs obtained from 8 patients revealed normal flora in all specimens. Leukocytes and platelets were normal or elevated.

In 87.7 % of our patients, no specific treatment was warranted, while in the remaining 13.3%, short courses of steroids were started.

HSP, also known as anaphylactoid purpura, is an immunologically mediated systemic vasculitis of small vessels affecting predominantly the skin, gastrointestinal tract, joints, and kidneys (5, 10).
A variety of other unusual manifestation can occur such as seizures, intracerebral hematoma, hemiplegia, cortical blindness pulmonary hemorrhage , myocardial infarction, pancreatitis, intramuscular hemorrhage, cholecystitis and hemorrhagic cystitis(1,4,5,7,10) .

Our data gave the usual pattern of presentation of the syndrome. Only one patient had multiple intracerebral hematomas and unfortunately died, another patient had scrotal edema in addition to the usual clinical manifestation.

The etiology of HSP is unknown, although there has been association with infectious agents especially group A B-hemolytic streptococci and less commonly measles, rubella, adenovirus, parvovirus, and mycoplasma (1, 11, 12).

The role of preceding upper respiratory tract infection (URTI) has been discussed frequently in relation to etiology; our figure of 46 % of those having URTI is lower than the 75 % reported in literature (13).

Proof of streptococcal infection was not present in our study because none of our 8 patients grew group A B-hemolytic streptococci in the throat swab cultures obtained and only 4 patients had antistreptolysin-O (ASO) titer > 200 IU/ml .

Hypersensitivity to food, drugs (penicillin, sulfonamides, allopurinol, propylthiouracil, and quinidine) and insect bites has also been postulated (2, 8, 14).

Cases have been reported following vaccination for typhoid, measles, cholera, and yellow fever (4).
Complement abnormalities have been described in association with HSP: C2 deficiency, homozygous null C4 phenotype and C4B deficiency, glomerular C3 and properdin deposition, low CH50 and properdin and raised C3d concentration in the acute phase of the disease have suggested complement activation (1, 15).

In our cases, serum complement (C3, C4) determinations were done in 5 patients and all were normal which failed to support a role of complement activation in HSP.

Regarding histopathology of the skin, the early changes are those of leukocytoclastic vasculitis with extravasations of erythrocytes. In the later stages the picture becomes less florid and mononuclear cells predominate with fibrin deposition and impaired fibrinolysis (14).

The direct immunofluorescence analysis evidenced vascular deposition of IgA and C3 in upper and mid dermis (5, 9).

Renal biopsy findings may be graded according to the classification of the international study of kidney disease in children (ISKDC) from 1-5 .

The primary lesion is endocapillary proliferative glomerulonephritis involving both endothelial and mesangial cells, but proliferation of extracapillary cells may result in crescent formation, immunofluorescense usually reveals mesangial IgA with IgG, C3, and fibrin(1).

There is widespread agreement that corticosteroids have a beneficial effect on gastrointestinal and central nervous system symptoms(4,5) . This was true in our study where the severity of abdominal pain was ameliorated in those patients receiving short courses of steroids and the response was fairly dramatic in a few.

Treatment with cyclophosphamides, plasmapheresis and azathioprine is .
controversial (4).

HSP is generally a benign disease, but recurrences are common occurring in up to half of the patients (8, 14).

The prognosis depends on the degree and severity of renal involvement. No relation was found between prognosis and age, streptococcal infection, type of presentation or relapse rates (14).
Death may occur during the acute phase of the disease as a result of bowel infarction, CNS involvement or renal disease (15) was seen in one of our patients who developed multiple intra-cerebral hematomas and unfortunately died.

In conclusion HSP is generally a self limited disease with an excellent overall prognosis.