Cutis
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Congenital fascial dystrophy is an uncommon disorder that may mimic scleroderma. The skin is bound down and feels "wood hard" on palpation of the trunk and extremities. ⋯ Flexion contractures result and other associated abnormalities may include developmental delay and mild hirsutism. We report a typical case to illustrate the clinical and pathologic findings of patients with this disorder.
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Although genital ulcers are a common manifestation of Behçet's disease, bleeding from these lesions is unusual. In this report, a patient with excessive bleeding from genital ulcers is presented. A 22-year-old man with recurrent oral and genital ulcers, erythema nodosum, folliculitis, positive pathergic reaction, uveitis, superior vena cava syndrome, and generalized lymphadenopathy fulfilled the diagnostic criteria of Behçet's disease. ⋯ Examination of a biopsy specimen obtained from the leg ulcer showed findings consistent with Kaposi-like acroangiodermatitis. Results of various radiologic investigations showed thrombosis of the inferior vena cava, peritesticular varicosities, and venous insufficiency of the lower extremities. We suggest that the bleeding was secondary to the extension of genital ulcers to the varicose veins.
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Case Reports
Intravenous cyclosporine therapy in the treatment of pyoderma gangrenosum secondary to Crohn's disease.
Pyoderma gangrenosum is a destructive, ulcerative skin condition often associated with systemic illnesses such as inflammatory bowel disease, myeloproliferative disorders, and the inflammatory arthritides. We present a patient with long-standing pyoderma gangrenosum associated with Crohn's disease. Multiple deep leg ulcerations were unresponsive over the course of several years to treatment with azathioprine, systemic corticosteroids, 6-mercaptopurine, and dapsone. The patient was hospitalized and treated with a ten-day course of intravenous cyclosporine therapy followed by outpatient oral cyclosporine and showed significant improvement.