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PEDIATRICS Vol. 103 No. 2 February 1999, pp. 500-502

EXPERIENCE AND REASON:
Metastatic Crohn's Disease Presenting as Chronic Perivulvar and Perirectal Ulcerations in an Adolescent Patient

The first 300 words of the full text of this article appear below.

Metastatic Crohn's disease (MCD) is a rare, granulomatous, extraintestinal manifestation of Crohn's disease (CD) with multiple clinical presentations.1 As such, there is often a delay in diagnosis and consequent therapeutic intervention. We describe MCD presenting as chronic perivulvar and perirectal ulcerations in an adolescent patient without history of gastrointestinal involvement. Additional investigation revealed active colorectal CD, and appropriate treatment was subsequently initiated.

    CASE REPORT

A 13-year-old African-American girl presented to her primary care physician with a 6-month history of hemorrhoids, constipation, perineal and perivulvar fissures, skin tags, and weight loss. She denied any nausea, vomiting, fever, cough, or night sweats. She had been previously in good health and was on no medications. Physical examination revealed multiple erythematous, indurated, tender papules and plaques, with granulomatous vegetations and ulcerations involving the inguinal, perivulvar, perineal, and perirectal regions. A few edematous, inflamed papillomas were noted in the perirectal area. There was no evidence of sinus tract or fistula formation. Mild tenderness of the lower abdominal region was observed on palpation, but the remainder of her physical examination was unremarkable. Serum electrolytes, liver transaminases, white blood cell count, platelet count, and urinalysis were within normal limits. Abnormal laboratory values included hemoglobin 10.5 µg/dL (normal values: 12-15 µg/dL), ferritin 5.6 ng/mL (normal values: 7-140 ng/mL), and serum albumin 2.9 g/100 mL (normal values, 3.5-5.5 g/100 mL). Results of chest radiography were normal, and a tuberculin skin test result was nonreactive.

On referral to pediatric gastroenterology, biopsies of friable, granulomatous, perivulvar tissue and an adjacent skin tag were performed. Histopathology of both specimens revealed ulcerated fibromembranous tissue, diffuse acute and chronic inflammation with a lymphohistiocytic infiltrate, and noncaseating granuloma formation with multinucleated giant cells consistent with MCD (Fig 1). A few of the foreign body type giant cells contained polarizable material and calcifications. PAS and Fite stain results were . . . [Full Text of this Article]