Crohn Disease-Associated Genital Edema : Case Report
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An adolescent boy presented to the dermatology clinic with a 3-year history of penile edema and scrotal swelling that had suddenly worsened. His history was notable for Crohn disease that was diagnosed 4 years previously and managed with adalimumab after treatment with prednisone, 6-mercaptopurine, and infliximab failed. There was no lymphadenopathy and no history of trauma, infections, or instrumentation. The patient denied any history of urinary tract infection symptoms. He mentioned a feeling of incomplete evacuation but did not have any other gastrointestinal symptoms at the time of presentation. A physical examination revealed scrotal and penile edema, perianal swelling, and perineal swelling (Figure). A biopsy specimen was obtained, and the results were compatible with extraintestinal Crohn disease.

The differential diagnosis for genital swelling is broad and may include angioedema; lymphatic obstruction; inflammatory skin conditions, such as hidradenitis suppurativa and contact dermatitis; fluid overload; neutrophilic dermatoses; sarcoidosis; typical and atypical infections; and extraintestinal Crohn disease. Extraintestinal or cutaneous Crohn disease should be suspected in the setting of otherwise unexplained genital edema, even in the absence of gastrointestinal symptoms. In approximately 25% of cases, Crohn disease can precede gastrointestinal involvement by months to years. Children and adolescents are reported more often than adults to present with extraintestinal Crohn disease in the absence of any gastrointestinal symptoms. Additionally, in patients with known gastrointestinal disease, extraintestinal Crohn disease is not necessarily associated with the short-term or long-term severity of their gastrointestinal symptoms.

Multidisciplinary care involving the treatment of the underlying inflammatory bowel disease is frequently effective; however, relapse is common. Treatment can be challenging, and it is suggested that management be individualized. A range of treatment options exists, including oral metronidazole, corticosteroids, physical or occupational therapy, compression, and reconstructive urologic surgery. In this case, the patient was treated with adalimumab and methotrexate combination therapy, daily topical tacrolimus to treat localized cutaneous inflammation associated with the edema, and a combination of compression shorts during the daytime and compression wraps at night. The patient experienced notable improvement of the genital swelling with this combination therapy approach.

Source: https://jamanetwork.com/journals/jamadermatology/fullarticle/2757503?widget=personalizedcontent&previousarticle=2763189
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