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Letterer-Siwe Disease. Presented by Dr. P. J. Koblenzer and I. J. Wolman.
The patient is a 5-month-old white girl, pale and obviously ill, who was well until age 2 months. Then she developed greasy scales on the scalp. At the age of 3 months, she developed a severe diaper rash and persistent bilateral otorrhea. For the past two months, the lesions now present on the trunk and limbs have gradually appeared.Dermatological examination reveals: (1) intertriginous dermatitis with a marked hemorrhagic component involving groin, axillae, neck, and retroauricular areas and both ears; (2) extensive crusted dermatitic eruption with numerous petechiae involving the entire scalp; (3) hemorrhagic papules 1-3 mm diameter involving trunk, arms, and buttocks.Medical examination reveals: hepatosplenomegaly, anemia, and bilateral otorrhea.
Hgb was 9.9 gm/100 ml; WBC, 9,250; neutrophils, 45%; reticulocytes, 12.5%; eosinophils, 3%; lymphocytes, 49%; monocytes, 31%; platelets, 170,000 cm; bleeding and clotting time, normal
PHILADELPHIA DERMATOLOGICAL SOCIETY. Arch Dermatol. 1964;90(3):337–339. doi:10.1001/archderm.1964.01600030087019
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