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11 January 2012 - Case of the Week #228
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Case of the Week #228
A healthy 14 year old boy presented with chronic, asymptomatic, recurrent scrotal and penile swelling of 2.5 years duration. He had no other signs or symptoms. All laboratory and radiological investigations were normal. A scrotal biopsy was obtained.
What is your diagnosis?
Granulomatous lymphangitis of the scrotum and penis
Granulomatous lymphangitis should be considered in the differential diagnosis of chronic idiopathic swelling of the genitalia, particularly in younger individuals. Most cases of granulomatous lymphangitis occur subsequent or concomitant with Crohn’s disease of the bowel, which some consider to be fundamentally a lymphocytic and granulomatous lymphangitis (Gut 2008;57:1). Granulomatous lymphangitis is also associated with orofacial granulomatosis (granulomatous cheilitis and Melkersson-Rosenthal syndrome) (J Cutan Pathol 2001;28:419).
Only a few cases of granulomatous lymphangitis are documented in children who never developed Crohn’s disease with bowel involvement. Pathogenesis of this isolated form is not clear. It could be a “form fruste” of Crohn’s disease, a bacterial / viral disorder or a distinct entity. Long term follow up is recommended to look for any evidence of Crohn's disease.
Treatment with topical and systemic steroids, metronidazole, sulfasalazine or immunosuppressive therapy is usually effective.
Nat Pernick, M.D., President
and Liz Parker, B.A., Associated Medical Editor
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