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Diagnosis: Cutaneous Crohn's Disease (Syn: Metastatic Crohn's Disease)
Histopathology: Necrotizing vasculitis with granulomatous features consistent with cutaneous Crohn's disease (Figs. 2 & 3).
Colonoscopy: Cobblestoning appearance in a skip pattern within the gastrointestinal tract suggestive of Crohn's disease.
Discussion:
Cutaneous disorders are commonly associated with Crohn's disease, reported in up to 15%. Perianal, perifistular, and peristomal inflammation are the most common cutaneous manifestations of Crohn's disease. Infrequently, patients have pyoderma, gangrenosum, erythema nodosum, and cutaneous polyarteritis nodosa. Rarely, patients may develop gramilomatous dermatitis at locations remote from the gastrointestinal tract, termed metastatic Crohn's disease. Few cases have been reported in the literature, but the condition may be more common as cutaneous lesions are often misdiagnosed.
Clinically, cutaneous Crohn's disease may manifest as nodules, plaques, or ulcers located on the extremities or in intertriginous areas. Lesions may arise during active or quiescent periods of gastrointestinal disease. Microscopically, cutaneous Crohn's disease is characterized by non caseating granulomas composed of collections of epithelioid and histiocytic giant cells surrounded by a scant rim of lymphocytes and plasma cells. The histologic differential diagnosis of granulomatous dermatitis includes sarcoidosis, fungal and mycobacterial infection, and foreign body reactions. These conditions may be excluded by clinical presentation, appropriate cultures, periodic acid-Schiff (PAS) stain, acid-fast stain, and polarized microscopy.
No definitive relationship exists between cutaneous lesions and intestinal symptoms. Treatment of gastrointestinal disease may or may not clear skin disease. Skin lesions may respond to intralesional and systemic corticosteroids, sulfasalazine, azathioprine, metronidazole, and curettage. The course of the lesions may be prolonged, but most lesions will resolve.
References:
1.Burgdorf W. Cutaneous manifestations of Crohn's disease. J Am Acad Dermatol(1981):5:689 - 95.
2. Lebwohl M, Fleischmajer R, Janowitz H, Present D, Prioleau PG. Metastatic Crohn's disease. J Am Acad Dermatol (1984):10: 3 3 - 38.
3. Levine N, Bangert J. Cutaneous Granulomatosis in Crohn's Disease. Arch Dermatol (1982): 118:1006-9.
4. Paller AS. Cutaneous changes associated with inflammatory bowel disease. Ped Dermatol (1986):3:439-45.
5. Sutphen JL, Cooper PH, Mackel SE, Nelson DL. Metastatic cutaneous Crohn's disease. Gastroenterol(1984):86:941-4.
My thanks to Drs. H. Kim, J. Yoon, and K. Forsman for their assistance in the prepariation of this case.
