OCTOBER 1998


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 History: This 57 year-old caucasian male presented with wide-spread red-purple papules. Lesions were first noticed on the tongue and oral mucosa in the third decade of life and subsequently appeared on the face, back, arms, palms, and to a lesser extent on the chest, abdomen, and lower extremities. He also suffers from recurrent epistaxis that began at 45 years of age and now occurs on a daily basis.

Review of systems is significant for frequent migraine headaches, worsening shortness-of-breath, dyspnea-on-exertion, and orthopnea.

Family History: Four out of six siblings have recurrent epistaxis and similar, albeit milder, skin findings than the patient. One sister died of "breathing-related problems"

Physical Examination: There were imultiple 1-3 mm nonblanchable violaceous papules on the face (Fig 1), lips (Fig 2), tongue, oral mucosa, back and arms. Telangectasias were present in the same distribution, some in assocation with the papules. The palms showed purple papules coalescing to plaques (Fig 3). Similar changes are noted on the soles. The chest, abdomen, and legs were relatively spared. Serum crust is adherent to the nasal mucosa.

Laboratory Values:

Hemoglobin: 9; Hematocrit: 28

Chest X-Ray: Multiple pulmonary nodules

Stool: Brown, guaiac positive




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