Letterer-Siwe病の1剖検例

AN AUTOPSY CASE OF LETTERER-SIWE'S DISEASE

　Letterer-Siwe病の1例を臨床的に観察し且つ，剖検所見を検討する機会を得たのでここに報告する。

　本疾患は本邦皮膚科領域では今日迄10数例の報告をみているが，乳幼児をおかし，全身症状を伴うため小児科をおとずれる例が多く，小児科領域では約40例に達する報告がある。

A 6-month-old boy of typical Letterer-Siwe's disease with various skin manifestions com-posed of papules of red and yellowish-red colors, crusts, erosions and bleeding spots was reported. The eruption started with fever and accompanied with bilateral cervical lymphad-enopathy, anemia and hepatosplenomegaly. Histologic section of the skin eruption showed a dense infiltrate of reticulum cells and pronounced bleeding in the upper dermis and atrophy of the epidermis.

Slight leukocytosis, anemia red blood cell count, 2,810,000 and thrombocytopenia were proved, but bleeding time and blood coagulation time were within normal lmits. Roentgeno-grams of the bones did not show any abnormalities.

In spite of treatmens with adrenocortical steroids, antibiotics and blood transfusion, the disease took an acute course to its termination after temporary remission of the symptoms.

Histologic specimens of the thymus, lymph nodeb, liver, spleen and intestines obtained by autopsy showed the pathologic picture in their lymph follicle which was composed of proliferation of reticulum cels with increased reticulum fibers and slight deposition of Su-dan III-positive substances in some parts, and proliferation of histiocytes with scanty reticulum fibers and dense deposition of Sudan III-positive substances in other parts.

A review on this disorder, Hand-Schuller-Christian's disease and eosinophilic granuloma of the bone was performed.