Letterer-Siwe Disease

Abstract

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.

Hand-Schuller-Christian disease with secondary cutaneous involvement

“…The introduction of electron microscopy has provided new information on the identity of the histiocytosis X cell. Birbeck (Langerhans) granules (Birbeck, Breathnach & Bverall, 1961) specific to Langerhans cells have been found in the mononuclear infiltrate (Basset & Turiaf, 1965;Vollum & Farquharson, 1974), and it is now generally accepted that histiocytosis X cells are proliferating Langerhans cells. Comma-shaped bodies similar to those described by Gianotti & Caputo (1971) are also commonly found.…”

Letterer-Siwe disease in the adult

“…* This case was briefly reported by Dr Alexander in discussion of a patient presented by Dr Vollum at the Royal Society of Medicine (Vollum & Farquharson, 1974).…”

“…There were oozing erythematous areas on the sides of the neck. * This patient was briefly mentioned in the discussion of the case presented by Dr Volium at the Royal Society of Medicine (Vollum & Farquharson, 1974).…”

“…* This case was presented by Dr Vollum at the Royal Society of Medicine (Vollum & Farquharson, 1974). Chest X-ray showed diffuse reticulate shadowing in the lungs.…”

“…In a recent review by Chevrant-Breton (1978) twenty-three cases were reported who were aged over 16 years or older. In all there were fourteen over 50 years of age and this number included the three cases described in detail here (Cases 2, 3 and 7, Table 2) (Vollum & Farquharson, 1974). The total number of cases in adults over 50 years of age may be only nineteen.…”