Langerhans cells in the epidermis at the sites of vaccina virus inoculation were studied with the electron microscope. The cells contained unusually increased numbers of the Langerhans cell granules. Such abnormal Langerhans cells have not been described except for in histiocytosis X. Vaccinia virus particles were found in the Langerhans cells, where they were located individually or embedded in the granular matrix or in lysosomes.

No abstract

In urine samples of patients with Fabry's disease (angiokeratoma corporis diffusum, ACD) mulberry cells appear, exhibiting the ‘Maltese cross’ with polarized light; they are said to be characteristic of this disease. However, their diagnostic significance has never been clearly defined. It is also well known that patients with this disease have electron‐dense periodical lamellar bodies in the cells of various organs. Several electron microscopic investigations have demonstrated such lamellar bodies in urine samples from hemizygous male patients, but it remains unclarified whether heterozygous female carriers excrete similar bodies in urine. We examined urine samples from a female carrier of ACD and two hemizygotes with the disease, as well as from three patients with nephrotic syndrome. With the light microscope, mulberry cells with ‘Maltese cross’ were detected in all six patients, suggesting that these cells are not diagnostic for ACD. With the electron microscope, concentric lamellar spheres were found in the heterozygous female carrier and two hemizygous males, but not in patients with nephrotic syndrome. This is the first report describing urinary laminated bodies in a female carrier of ACD. It is concluded that the demonstration of mulberry cells with ‘Maltese cross’ as well as concentric lamellar bodies in urine is helpful in detecting female carriers of ACD and hemizygotes as young as 7 years of age, before they exhibit definite clinical manifestations.

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In a case of infantile digital fibromatosis in a 5-month-old female baby, there were lesions on the digits, and later a tumor recurred on the sole. From histological, histochemical and electron microscopic testing, it is suggested that the specific inclusion was composed of peptide with little or no sugar or nucleic acid content and that it was an accumulation of "immature" tropocollagen. A brief review of the literature is presented.