Case 21-2000

Abstract

A 13-year-old boy was admitted to the hospital three years and eight months before the diagnostic procedure because of abdominal pain.Periumbilical pain had begun the day before the initial admission, after the patient had eaten cookies and an apple. He slept poorly that night, and by the next morning the pain had shifted to the right lower quadrant of the abdomen. During that day the temperature remained normal. In midafternoon he vomited 240 ml of red liquid after consuming a soft drink and was brought to this hospital. There was no history of fever, sweats, chills, nausea, vomiting, urinary frequency, dysuria, or hematuria.The temperature was 35°C, the pulse was 110, and the respirations were 18. The blood pressure was 110/60 mm Hg.Physical examination showed tenderness in the right lower abdominal quadrant, without signs of peritoneal irritation. The results of a rectal examination were normal. The external genitalia appeared normal. The urine was positive (+++) for ketones. Laboratory tests were performed (Tables 1 and 2). Abdominal radiographs revealed fecal debris in the colon. By the second hospital day, the pain had disappeared, and the findings on examination of the abdomen were normal. The patient was able to eat and was discharged home.Eight months later, his penis and scrotum became swollen. Treatment with antibiotics and corticosteroid ointment did not reduce the swelling. The patient returned to this hospital. He had no history of trauma, sexual contact, pain in the genital area, or travel to exotic areas, and there was no family history of lymphedema. Examination revealed soft, puffy swelling at the base of the penis, without evidence of inflammation. There was no edema of the legs. A retrograde urographic study with a voiding cystourethrogram showed slight narrowing of the urethral meatus without proximal dilatation, posterior urethral valves, and slight trabeculation of the bladder wall. No ureteral reflux was detected, and only a small residual volume of urine was present after voiding. Cystoscopic examination revealed narrowing of the urethral meatus and moderate trabeculation of the bladder. A small bladder diverticulum was excised, and urethral dilation and a meatotomy were performed.Examination two weeks later revealed increased edema in the area of the prepuce and the median raphe of the scrotum. Nonpurpuric cutaneous lesions were noted on the thighs. One month later, the edema was unchanged. A renal ultrasonographic study showed extensive soft-tissue swelling around the corpora cavernosum; the kidneys and bladder appeared normal. A computed tomographic (CT) scan of the abdo-*To convert the values for iron and iron-binding capacity to micromoles per liter, multiply by 0.1791.

Successful Treatment of Metastatic Crohn Disease With Infliximab

“…The symptom of isolated genital edema in a boy has a differential diagnosis (Table 1) that normally would not include metastatic Crohn disease. In this case, other con- No response, less gastrointestinal complaint, but no improvement in lymphoedema (which was already present for 3 y) very similar to this have been described (2-5,7), but all patients had bowel symptoms (and gastrointestinal inflammation) either before (2), during (3,7), or years after the onset of genital symptoms (4,5). In an excellent review and additional case reports, Ploysangam et al (7) described 80 cases of cutaneous Crohn disease, 14 of which were in children.…”

“…Positive signs of Crohn disease in this case were the characteristic granulomatous infiltrate of the scrotal skin and the presence of subtle perianal lesions, such as a tag and a minor fissure. Four cases very similar to this have been described (2–5,7), but all patients had bowel symptoms (and gastrointestinal inflammation) either before (2), during (3,7), or years after the onset of genital symptoms (4,5). In an excellent review and additional case reports, Ploysangam et al (7) described 80 cases of cutaneous Crohn disease, 14 of which were in children.…”

“…Metastatic Crohn disease in the skin of male genitals presents differently in children (2–5) than in adults (8–10). Children have genital edema, whereas adult men have ulceration of the penile or scrotal skin, often in conjunction with perianal disease, such as extensive fistulae.…”

“…Two thirds of the children had genital involvement, and in 11 of the children, cutaneous Crohn disease preceded intestinal Crohn disease by weeks to 6 years. Metastatic Crohn disease in the skin of male genitals presents differently in children (2)(3)(4)(5) than in adults (8)(9)(10). Children have genital edema, whereas adult men have ulceration of the penile or scrotal skin, often in conjunction with perianal disease, such as extensive fistulae.…”

“…Dermatologic manifestations, reported in 1% to 10% of children with Crohn disease (1), can occur without specific histopathologic abnormalities (i.e., erythema nodosum, pyoderma gangrenosum) or may be referred to as metastatic Crohn disease, with characteristic granulomatous skin lesions affecting the groins, the male and female genitalia, or submammary regions. Several case reports describe children with isolated symptoms of the penile and scrotal skin (2)(3)(4)(5)7). Medical treatment of this rare condition has been of limited value.…”