Effectiveness of infliximab in the treatment of refractory perineal cutaneous Crohn disease.

Abstract

A 47-year-old woman presented with a 19-year history of Crohn disease, complicated by ileocolitis, fistulous tract formation, and multiple cutaneous manifestations, including nonfistulous erosions, ulcerations, and nodules involving her perineum and inner thighs. Her clinical course included several surgical reconstructive procedures, azathioprine therapy (100-300 mg/d orally for over 5 years), and long-term courses of intravenous methylprednisolone and oral prednisone (up to 100 mg/d), ciprofloxacin, and metronidazole. All of these therapeutic interventions failed to achieve complete and prolonged remission of her intestinal and cutaneous disease. Her ileocolitis had been severe throughout her disease course. Fistulas, both enterocutaneous and enterovaginal, had been recurrent, slow to heal, and characterized by profuse yellow fibrinous drainage. Nonfistulous cutaneous manifestations began to emerge as a prominent feature after approximately 10 years of active Crohn disease. Initial complaints of persistent nonhealing erosions and ulcerations in her perineum were localized mostly to her posterior gluteal region, vulva, and inner thighs. The patient later developed large painful nodules in her perineum. These ulcerations and nodules did not communicate with underlying fistulous tracts. Histologic examination of the skin lesions showed noncaseating granulomatous inflammation with hyperkeratosis and irregular acanthosis. A prominent diffuse dermal and subcutaneous granulomatous inflammation composed of giant cells, neutrophils, lymphocytes, and eosinophils was present. The skin specimens were stained with Brown and Brenn, Giemsa, Periodic Acid‐Schiff, Ziehl-Nielsen, and Fite stains and the findings were negative; cultures for fungi, bacteria, and mycobateria of these skin specimens were repeatedly negative. The findings from examination under polarized light were negative for refractile elements. These were all findings consistent with cutaneous Crohn disease. DIAGNOSTIC CHALLENGE