Letterer-Siwe's Disease (Reticuloendotheliosis). Presented by Dr. Irving J. Wolman (by invitation).
A white boy, aged 4 years, presented a persistent cutaneous rash and crusted lesions on the scalp. The first symptom was pallor, noted at the age of 17 months. At the same time, seborrhea, rash, and "external otitis" began. At 20 months the child developed swelling of the axillary lymph nodes. Medical examination revealed many areas of bone decalcification; their progress has been arrested by local radiotherapy. The patient's nutrition is good so long as adrenal steroids are given. Early blood cell counts ranged from 9.0 to 12.0 gm. hemoglobin, 10,000 to 15,000 leukocytes; 60% to 75% polymorphonuclear leukocytes; no eosinophilia; no abnormal cells.
The report on a biopsy specimen from an axillary lymph node was "reticuloendotheliosis." The report on a specimen from the skin was as follows: "Many histiocytes in subepidermal tissues; these had
Flood JM, Luscombe HA. PHILADELPHIA DERMATOLOGICAL SOCIETY. AMA Arch Derm. 1959;79(1):123–126. doi:https://doi.org/10.1001/archderm.1959.01560130125023
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