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This 22-year-old woman complained of difficulty smiling, weakness of her hands, and bumps on her skin of two months’ duration.On examination, she had bilateral facial weakness (more prominent on the left), a left abducens weakness, and bilateral ulnar palsies. She also had red,indurated papular lesions on her left hypothenar eminence and abutting her nose. The skin lesions were anesthetic and showed granulomatous inflammation onbiopsy.
The clinical findings in this patient initially suggested sarcoidosis, then leprosy. But after extensive study at two universitymedical centers and the Public Health Service Hospital at Carville, Louisiana, the final diagnosis was lymphomatoid granulomatosis—a disease with featuresresembling those of lymphoma and Wegener’s granulomatosis. Histologically, the characteristic finding is a granulomatous process with a polymorphouslymphoreticular infiltration, angiocentric and angiodestructive in nature, with atypical and occasionally bizarre lymphohistiocytic cells.
Although virtually any organ can be affected by this disease, pulmonary, neurologic, and cutaneous manifestations predominate.No consistently effective therapy has been established, and the prognosis is poor.
In the patient shown, the skin lesions resolved with corticosteroid therapy, but the neurologic difficulties worsened. And aftera year of illness, the patient had become paraplegic. Curiously, no evidence of pulmonary disease ever emerged during that time.
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