Jessica Metakes ARNP, NP-C, FNP-BC
Blue Ocean Dermatology
Henoch-Schonlein purpura (HSP) is a condition that usually causes a rash that looks like tiny raised bruises and is described as “palpable purpura”. The condition was first recognized in 1837 by Johann Lukas Schonlein, and called ‘purpura rheumatica’ due to the purpura and joint pain seen in children affected by the condition. A student of Schonlein’s, Eduard Heinrich Henoch recognized the significant gastrointestinal and renal involvement associated with the condition and published the first case series in 1867.
HSP, also known as Immunoglobulin A vasculitis (IgAV),
is the most common form of vasculitis in children with an estimated incidence rate of approximately 150 cases per million. It can occur in adults, but the incidence rate is much lower with approximately 5-15 cases per million. In children, HSP affects both sexes equally, while in adults there is predominance for males. It frequently presents 1-2 weeks following an upper respiratory infection, particularly in children, and likewise follows a seasonal pattern, with peak incidence during the winter months.
The initial symptom of HSP/IgAV is:
usually the palpable purpura rash, but often (approximately 75% of patients) include arthralgias/arthritis (joint pain/joint swelling), and colicky abdominal pain, which may be accompanied by nausea and/or vomiting. Fever occurs in approximately 40% of children, but less frequently in adults. The skin lesions usually have a symmetrical distribution with a predilection for the lower extremities and buttocks. Lesions can also occur on the trunk, upper extremities, and face, and typically resolve over a period of several weeks to months.
Renal involvement is common, but typically the effects of HSP/IgAV on the kidneys are mild, and self-limited, including microscopic hematuria and proteinuria (blood and/or protein in the urine). Less than 5% of patients will develop long-term kidney impairment, but of those, adults are more likely, and particularly those with the purpuric rash above the waist, fever, and elevated erythrocyte sedimentation rate (ESR).
Diagnosis is typically based on:
clinical presentation, however, if diagnosis is uncertain, additional testing may be needed. A skin biopsy of the rash may be performed, and less often a biopsy sample of the kidneys may be warranted if there is suspected renal involvement. There are no diagnostic laboratory tests for HSP/IgAV however; it may be necessary to perform additional testing to support the diagnosis and manage treatment. Laboratory tests that may be ordered include a complete blood count, coagulation studies, a metabolic profile, erythrocyte sedimentation rate, and urinalysis.
Treatment for HSP/IgAV is:
mainly supportive, because the cutaneous and musculoskeletal symptoms are generally self-limited, and resolve over the course of a few weeks to months. Management depends on the severity of the associated renal involvement, since renal vasculitis could potentially (but rarely) develop into chronic renal impairment. Treatment primarily involves increasing fluid intake, resting, and management of pain with NSAIDs such as naproxen (Aleve) or ibuprofen (Advil). However, pain and swelling unrelieved by the NSAIDs may need systemic/oral steroid therapy treatment, such as Prednisone. If you or your child develops symptoms of HSP/IgAV, it is imperative to seek medical attention with your care provider for proper evaluation, testing, and treatment.
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Bolognia, J. L., Jorizzo, J. L., Rapini, R. P., Callen, J. P., Horn, T. D., Mancini, A. J., … Stingl Mary S Stone, G. (2008). Dermatology (2nd ed.). Spain: Mosby Elsevier.
Dedeoglu, F., & Kim, S. (2017). IgA Vasculitis (Henoch-Schonlein Purpura): Clinical manifestations and diagnosis. Retrieved from https://www.uptodate.com