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Henoch-Schönlein Purpura – an unusual presentation (clinical case)
Pediatric Rheumatologyvolume 6, Article number: P275 (2008)
Henoch-Schönlein Purpura (HSP) is the most frequent children's vasculitis, predominant in male gender and more often between the ages of 2 and 14 years.
The etiology is still unknown but generally it has a good outcome.
The first event is typically a lower limb palpable purpura, with involvement of the gut or kidney. The renal disease usually appears in the first month, progressing to renal insufficiency in about 5% to 20%.
The authors present a case of a male toddler, that soon after birth, began with intermittent episodes of diffuse erythematosus-maculopapular rash on the face, trunk and upper limbs. These cutaneous manifestation where associated with transitory hematologic alterations and elevation of serologic IgA, which resolved after a short period of corticoids. Skin biopsy revealed vasculitis.
Ten years later, the child complained about unspecific polyarthralgia and was referred to Pediatric Rheumatology. He was normotense, without signs of arthritis, but with diffuse erythematosus-maculopapular rash on the face, trunk and upper limbs and macroscopic hematuria. Kidney biopsy revealed IgA nephritis and the diagnosis of HSP could be made. The treatment began using corticoid and ACE inhibitors, because of persistent microalbuminuria.
He maintains the renal function stabilized, but complains of recidivant cutaneous lesions that require higher doses of corticoid.
This clinical case relevance resides in the fact that these recidivant cutaneous lesions are found in atypical regions and that kidney involvement begun at a rather late period, when generally is revealed in the first months of the disease onset.