Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Crohn’s Disease in the Scrotum

  • Manuel NistalEmail author
  • Pilar González-Peramato
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_4796-1

Definition

Crohn’s disease is a chronic inflammatory intestinal pathology which can be associated to different extra-digestive manifestations such as ano-genital granulomatosis, inguinal fistula with recurrent penile, and scrotal lymphedema.

Clinical Features

Recurrent genital lymphedema is associated or not with fever and gastrointestinal symptoms. Other symptoms are funiculitis or orchioepididymitis in patients with long-standing Crohn’s disease. Other times, the first manifestation of Crohn’s disease are inguinal fistulas (Reitsma et al. 2012), and especially in children, genital edema (Barrick et al. 2016).
  • Incidence

    Genitourinary complications occur in 5–20% of patients with active Crohn’s disease. Isolated scrotal and penile lymphoedema as the presenting symptom of Crohn’s disease is quite unusual.

  • Age

    At any age

  • Sex

    Male

  • Treatment

    Treatment with corticosteroids and azathioprine results in significant decrease of inflammatory genital lymphedema. Fistulous tracts should be surgically removed. If the affectation involves paratesticular structures with epididymis and/or testis, orchiectomy can be indicated.

  • Outcome

    Most patients with penile and scrotal lymphedema initially respond well to steroid therapy but relapse is common. Similar results are obtained with azathioprine. Good results are also obtained with antitumor necrosis factor [TNF] therapy (Alexakis et al. 2017).

Macroscopy

Grossly, lesion causes scrotal swelling and marked scrotal wall thickening.

Microscopy

Removed tissues show a background of chronic inflammation, small microabscesses of geographic contours, and epithelioid granulomas (Fig. 1). In these noncaseating granulomas, multinucleated giant cells of both Langhans and foreign body type are observed (Garny de la Rivière et al. 2015).
Fig. 1

Crohn’s disease. Paratesticular tissue with non-necrotizing granulomas formed by histiocytic cells and lymphocytes

Differential Diagnosis

Acquired lymphedema of the genitals is more frequent in elderly individuals as a consequence of pelvic/abdomen malignancy or its therapy or worldwide due to filariasis than in childhood (Murphy et al. 2001). Scrotal swelling in young boys is secondary to testicular torsion, orchioepididymitis, or idiopathic. But it can also be the first symptom of Crohn’s disease. The diagnosis of pediatric genitourinary Crohn’s disease is made taking into account the clinical data and the histological study. The medical history and physical examination can reveal a history of benign gastrointestinal complaints and perianal skin tags and fissures. Histologically, the presence of noncaseating granulomas similar to those of sarcoidosis would be required for a differential diagnosis of laboratory evaluation (angiotensin-converting enzyme and lysozyme to assess sarcoidosis). To rule out mycobacterial disease apart from the caseating morphology of granulomas, culture and polymerase chain reaction to identify mycobacterial disease as well as chest radiography (Weinberg et al. 2012) is useful.

References and Further Reading

  1. Alexakis, C., Gordon, K., Mellor, R., Chong, H., Mortimer, P., & Pollok, R. (2017). Ano-genital granulomatosis and Crohn’s disease: A case series of males presenting with genital lymphoedema. Journal of Crohn’s & Colitis, 11, 454–459.CrossRefGoogle Scholar
  2. Barrick, B. J., Tollefson, M. M., Schoch, J. J., McEvoy, M. T., Hand, J. L., Wieland, C. N., & Davis, D. M. (2016). Penile and scrotal swelling: An underrecognized presentation of Crohn’s disease. Pediatric Dermatology, 33, 172–177.CrossRefGoogle Scholar
  3. Garny de La Rivière, C., Caudron, A., Heyman, B., & Lok, C. (2015). Genital lymphedema associated with Crohn’s disease. La Revue de Médecine Interne, 36, 291–293.CrossRefGoogle Scholar
  4. Murphy, M. J., Kogan, B., & Carlson, J. A. (2001). Granulomatous lymphangitis of the scrotum and penis. Report of a case and review of the literature of genital swelling with sarcoidal granulomatous inflammation. Journal of Cutaneous Pathology, 28, 419–424.CrossRefGoogle Scholar
  5. Reitsma, W., Wiegman, M. J., & Damstra, R. J. (2012). Penile and scrotal lymphedema as an unusual presentation of Crohn’s disease: Case report and review of the literature. Lymphology, 45, 37–41.PubMedGoogle Scholar
  6. Weinberg, A. E., Hazard, F. K., & Hsieh, M. H. (2012). A case of genitourinary Crohn’s disease. Urology, 80, 1132–1134.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Anatomy, Histology and NeuroscienceUniversidad Autónoma de MadridMadridSpain
  2. 2.Department of PathologyUniversidad Autónoma de MadridMadridSpain
  3. 3.Department of PathologyUniversity Hospital La PazMadridSpain