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Background
Henoch-Schönlein purpura (HSP) is the commonest vasculitis in children. It is a systemic leucocytoclastic vasculitis of the small vessels with perivascular deposition of immunoglobulin A (IgA) immune-complexes leading to inflammation and necrosis of arterioles, capillaries and postcapillary venules. It mainly affects the small vessels of the skin, joints, gastrointestinal tract and, more rarely, kidneys, brain, lungs and genitalia. Clinically it is characterised by a palpable purpuric rash with a typical distribution on the buttocks, thighs and lower legs.
Scrotal involvement, consisting mainly of scrotal swelling and tenderness, may occur in HSP, usually unilaterally, with a mean prevalence of 11.6% (ranging from 2% to 38%). 1 2 Penile involvement is much rarer, with few cases reported in detail in the literature, as far as we could ascertain. 3-9 It may occur in isolation or in association with scrotal involvement. 1
We describe a child with HSP who developed penile involvement. We review the literature of all the cases reported in detail, in order to highlight useful features of the clinical presentation, management and prognosis of this rare manifestation.
Case presentation
Six months following an episode of Henoch-Schnölein purpura (HSP), a 4-year-old boy presented with an acute swelling of the penis over the previous few hours, with no preceding illnesses or other symptoms. There was no history of trauma or penile discharge, the swelling was painless and there was no difficulty in passing urine, and the scrotum looked normal. With the exception of a palpable erythaematous rash on the elbows and abdomen, he was otherwise well and had no symptoms in his joints.
Although on examination he had a widespread palpable erythaematous maculopapular rash on his elbows and abdomen, and marked swelling of the uncircumcised penis associated with discolouration of the overlying skin, he appeared well and was apyrexial, haemodynamically stable and well perfused. Over the next 24 h, the maculopapular rash became purpuric and the boy developed a purpuric rash with swelling, a bruise and tenderness over the penis, the upper scrotum and the suprapubic area ( figure 1 ), associated with dysuria. It was not possible to elicit specific testicular tenderness as the whole genital area was swollen and tender.
Investigations
Doppler ultrasound studies demonstrated increased blood flow to the testicles, with normal flow...