Letterer-Siwe disease in a young adult

Ward, A. Milford

doi:10.1111/j.1365-2133.1973.tb02981.x

Cited by 5 publications

(1 citation statement)

References 1 publication

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“…Doede & Rappaport (1967) in their series of 96 cases suggest that an onset below the age of 3 should be one of the diagnostic criteria. Shahani & Ward (1973) recently described a case in a girl of 15. There are references in the literature to perhaps 6 other cases in older patients (Enriquez et al 1967, Lichenstein 1953) but the details are too incomplete for accurate diagnosis.…”

Section: Commentmentioning

confidence: 99%

Letterer-Siwe Disease

Vollum

Farquharson

1974

Proceedings of the Royal Society of Medicine

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R C, man aged 74 History: Shortness of breath for eighteen months which has become persistent and increasingly severe. The patient has felt distended after meals * and lost a stone (6.4 kg) in weight. For two months he has had a rash on the trunk and swelling of the ankles. On examination: Over the trunk, axille and upper arms there are maculopapular, mainly follicular purplish-brown lesions which are slightly scaly and fade on pressure. Some purpura on the forearms. Spleen enlarged 5 flngerbreadths, liver 2 flngerbreadths. No lymphadenopathy. Pitting cedema of ankles.

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Section: Commentmentioning

confidence: 99%

Letterer-Siwe Disease

Vollum

Farquharson

1974

Proceedings of the Royal Society of Medicine

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Primary Cutaneous Langerhans Cell Histiocytosis in an Adult

Lichtenwald

Jakubovic²,

Rosenthal³

1991

Arch Dermatol

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An unusual adult case of Langerhans cell histiocytosis is presented. The case initially mimicked granuloma annulare, both clinically and histologically. The patient subsequently had development of extensive cutaneous involvement that pathologically revealed a diffuse infiltrate of CD1-positive histiocytic cells containing Langerhans granules. Extensive investigations failed to detect systemic involvement. The patient's cutaneous eruption did not respond to various therapeutic interventions, including phototherapy with oral psoralen with long-wave UV radiation in the A range (PUVA) and systemic vinblastine sulfate. Marked but temporary clinical and histologic improvement was achieved with total body electron beam radiotherapy. The nosology of this case is discussed in the context of the various histiocytic proliferative disorders.

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