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E-case #14 Kathy Hurlburt, M.D. A 35 yo white male with a several year history of occasional short episodes of crampy abdominal pain associated with diarrhea, now presents with a painful, necrotic ulcer on his left lower extremity. He states the lesion began as a hemorragic pustule after bumping it on a coffee table two weeks ago. He denies fever/chills, nausea/vomiting. He has no history of skin disorders, he denies any other skin lesions, and has no other significant past medical history. His physical exam is significant for a large (5 x 6 cm), purulent ulcer on his anterior left lower extremity just above his knee. Its borders are irregular and raised, there is pus draining from the center, and it has a halo of erythema around the advancing borders. Questions: What is the differential diagnosis for this skin condition? With what other conditions is this skin disease associated? What are other extraintestinal manifestations of his primary disease? How are this and other extraintestinal manifestations of his primary disease treated? Discussion of Ecase #14: DDx= Progressive synergistic gangrene, ecthyma gangrenosum, atypical mycobacterial infection, clostridial infection, deep mycoses, amebiasis, pemphigus vulgaris, stasis ulcers, and Wegener's granulomatosis. Pyoderma Gangrenosa and other Dermatologic Manifestations of Inflammatory Bowel Disease: Extraintestinal manifestations are common in ulcerative collitis and crohn's disease, and may even be heralding symptoms. These conditions are broadly categorized into: rheumatologic, ophthamologic, and dermatologic. The incidence of skin conditions is estimated to be 15-20% in Crohn/s and 10% in UC. The dermatologic manifestations are further subclassified as"specific lesions," including perianal fissures and metastatic Crohn's. These lesions' severity parallels that of the disease activity of the bowel and have the histologic appearance of granulomas. A second group of dermatologic manifestations of IBD include erythema nodosum and pyoderma gangrenosum. These may occur as a result of malabsorption or drug therapy. They are both found to occur in other diseases. Erythema nodosum is an acute inflammatory/immunologic reaction pattern characterized by the appearance of painful, tender nodules on the anterior aspects of the lower legs. EN has been associated with infectious agents such as primary TB (mostly in children),coccidioidomycosis, histoplasmosis, beta-hemolytic streptococcus, Yersinia organisms, and leprosy. Drugs such as sulfonamides and oral contraceptives have also been implicated. Sarcoidosis and Behcet's are also known to be associated diseases with EN. About 40% of EN is idiopathic. Treatment of EN includes controlling IBD, bed rest, NSAIDS, corticosteroids, potassium iodide, dapsone, or colchicine. Pyoderma gangrenosa is a rapidly evolving, chronic, and severely debilitating skin disease. It has an acute onset with a painful hemorrhagic pustule of nodule de novo or following minimal trauma. Laboratory data, such as a CBC or ESR are variably elevated with PG. Biopsies are not diagnostic, showing only neutrophilic inflammation. Diagnosis of is based on clinical findings and course of illness. Distribution, in order of prevalence, includes lower extremities, buttocks, abdomen and face. Up to 50% of PG occurs without associated disease. The remainder of cases is associated with large and small bowel disease, diverticulosis, arthritis, paraproteinemia and myeloma, leukemia, and Behcet's syndrome. Untreated, the course may last for months to years. Ulcerations may extend rapidly within a few days or slowly. Healing may occur centrally with peripheral extension. New ulcers may appear as older lesions resolve. Treatment of PG also includes controlling the underlying bowel disease. High doses of oral corticosteroids or pulse doses of IV corticosteroids may be required. Intralesional triamcinolone may also be effective. A third group of skin conditions is loosely associated with IBD, the dermatoses. These include bullous diseases (such as bullous pemphigoid, linear IgA bullous, and epidermolysis bullosa acquista) and acneiform eruptions. This group is most closely linked to ulcerative colitis. References:
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