Henoch-Schonlein purpura is a common, self-limited generalized leukocytoclastic vasculitis. Vasculitis involves primarily small vessels and is mediated by immune complexes produced through IgA and the alternate complement pathways. A variety of infectious and noninfectious stimuli appear to precipitate the immunologic mechanisms of the vasculitis.
The disorder is classically characterized by the appearance of a dermovasculitis with a propensity for the lower trunk, buttocks, perineum, and lower extremities, as is characteristic of generalized vasculitides ( Fig 132-8). In addition, the vasculitis includes involvement of the glomeruli, with the subsequent development of hematuria and with the potential for long-term renal sequelae. Involvement of the bowel wall causes recurrent colicky abdominal pain and frequently leads to the development of melena or hematochezia. Eight percent of children with severe abdominal colic caused by Henoch-Schonlein purpura experience massive gastrointestinal hemorrhage or intussusception. Arthritis, or, more specifically, a polymigratory periarticulitis, occurs in most affected children. Rarely, massive pulmonary hemorrhage can occur.
FIG. 132-8. Henoch-Schonlein purpura. Purpuric lesions of dependent body areas, particularly on flanks, buttocks, and lower extremities.
The clinical diagnosis is not usually obscure in those children manifesting all or several characteristics of the disorder. The child with hematuria, abdominal pain, a history of migratory periarthritis, and a palpable, purpuric tender rash on the buttock and lower extremity is not a diagnostic dilemma. Difficulty ensues upon the evaluation of a child manifesting exclusively abdominal colic or apparent arthritis.
Care of the child with suspected Henoch-Schonlein purpura in the emergency department consists of establishing the diagnosis or maintaining a high index of suspicion for the diagnosis. Otherwise, emergency care is entirely supportive. Consideration of specific laboratory or imaging studies is influenced by the specific symptoms manifested by the child. For example, the child presenting exclusively with hematuria and abdominal pain may require a urine analysis and urine culture, a kidney-ureter-bladder abdominal radiograph, assessment of renal function, and a complete blood count. Such circumstances might even serve as an indication for a renal ultrasound or an intravenous pyelogram if the clinician suspects ureterolithiasis as the cause of the child's symptoms. Similarly, a child with migratory polyarthritis might reasonably be expected to undergo an evaluation of arthritis, including plain radiographs of affected joints, complete blood count, rheumatoid factor titer, antinuclear antibody titer (ANA) and complement levels. In summary, the diagnostic evaluation in the emergency department is often influenced by the need to exclude other disease processes that share common features with this generalized vasculitis. When the entire clinical picture presents itself, however, the extent of the diagnostic workup is much reduced.
Children with Henoch-Schonlein purpura are admitted to the hospital when the diagnosis is in doubt, for observation and control of abdominal pain, for monitoring of renal function, and for fluid hydration in the setting of recurrent emesis. Children with extremely mild symptoms can be safely and expectantly observed as outpatients, as long as an experienced primary care provider is available. Arthritis, when present as an isolated symptom, is usually easily controlled with aspirin. Prednisone is not utilized in the management of arthritis when it represents the child's only symptom of active vasculitis. The child's primary care physician maintains contact with the family on a daily basis and evaluates the child in the office at frequent intervals in the initial weeks following the establishment of the diagnosis. Particular attention is directed to the development of symptoms suggestive of abdominal colic, gastrointestinal hemorrhage, intussusception, and the development of chronic renal disease. Chronic renal sequelae are reported in approximately Z to 9 percent of children with Henoch-Schonlein purpura and do not respond well to glucocorticoid therapy.
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