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Letterer-Siwe Disease. Presented by Harold Price and Ellis J. Seligman.
The patient was well until 11 months of age when he developed bilateral ear infections and a papular petechial eruption with greasy scales involving the scalp and axilla. The eruption slowly spread to involve the trunk, neck, and groin. The lesions were quite pruritic and were treated unsuccessfully with multiple preparations (tars, steroid, and sulfur ointments as well as systemic antibiotics). At age 18 months, the child was noted to have increasing abdominal size and firmness. One month prior to admission the mother noted that the child's stool was yellowishwhite, foul-smelling, and painful to pass. The patient has recently developed scleral icterus and darkening of the urine. One week prior to admission the child had an apparent grand mal seizure.
The patient weighed 13.6 kg (30 lb) at 1 year of age and at present weighs
THE LOS ANGELES DERMATOLOGICAL SOCIETY AND THE METROPOLTAN DERMATOLOGICAL SOCIETY OF LOS ANGELES. Arch Dermatol. 1964;90(3):366–368. doi:10.1001/archderm.1964.01600030116029
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