Case: A 19-year-old male is admitted to the ICU with severe scrotal pain. Ultrasound reports read marked edema of the scrotal skin and contents with intact vascular flow in the testes, and acute torsion is ruled out. Patient reports having been told in childhood that he has some kind of vascular disease, but he does not remember.
Answer: Immunoglobulin A (IgA) vasculitis
Acute scrotal pain can be the presenting symptom of IgA vasculitis, also known as Henoch-Schönlein purpura. Although the scrotum is mostly involved, the ureter, bladder, prostate, testicle, and peniscan also be affected.
IgA vasculitis is a systemic vasculitis characterized by a tetrad of:
- Palpable purpura in patients with neither thrombocytopenia nor coagulopathy
- Arthritis/arthralgia
- Abdominal pain (+/- GI bleed)
- Kidney disease
It is usually a disease of children, but adults may be affected too. The differential diagnosis for torsion is hard to make solely on clinical exam. Ultrasound is the key, and is usually described as given in the question. Another clue to the diagnosis is its seasonal preference! It occurs primarily in the fall, winter, and spring but rarely in the summer. Probable explanations are its association with infections, mostly URI by Streptococcus. Besides infections such as RSV, influenza, and norovirus, vaccinations and insect bites have also been the cause. Other associations have also been described, such as familial Mediterranean fever.
Treatment is usually supportive and involves treating the underlying cause. In severe cases, steroids or immunosuppressants can be used.
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References:
1. Dalpiaz A, Schwamb R, Miao Y, et al. Urological Manifestations of Henoch-Schonlein Purpura: A Review. Curr Urol 2015; 8:66.
2. Balslev T. [Schoenlein-Henoch syndrome with acute scrotal condition simulating torsion of the testis]. Ugeskr Laeger 1990; 152:678.
3. Buscatti IM, Abrão HM, Kozu K, et al. Characterization of scrotal involvement in children and adolescents with IgA vasculitis. Adv Rheumatol 2018; 58:38.
4. Parums DV. A Review of IgA Vasculitis (Henoch-Schönlein Purpura) Past, Present, and Future. Med Sci Monit. 2024 Jan 28;30:e943912. doi: 10.12659/MSM.943912. PMID: 38281080; PMCID: PMC10832303.
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