Two children, one born prematurely and the other born at full term developed acrodermatitis enteropathica due to marginal or low levels of zinc in their mothers' breast milk. Skin from both patients was studied with light and electron microscopy. The most characteristic light microscopic features were parakeratosis, absence of the granular layer, and pallor of the upper epidermal cells. Normal flattening of the upper Malphighian cells did not occur. Electron microscopic examination revealed that keratohyalin was decreased to absent, and the upper malpighian cells were edematous with vacuoles and large numbers of ribosomes, but small numbers of tonofilaments. Large amounts of keratinosome-derived-lamellae were found in the intercellular spaces in the keratinization area. The keratinosome-derived-lamellae were focally intermingled with opaque lipid plaques or myelin figures, probably derived from keratinosomes. The electron microscopic findings show abnormal keratinization and suggest that it is related to a disturbance of keratinosome metabolism due to zinc deficiency.
A great variety of skin manifestations and the frequent occurrence of cold sensitivity, vascular symptoms with peripheral painful ulcerations, headache of migraine type, and muscle and joint symptoms are described in a series of 12 patients with an overlap syndrome compatible with mixed connective tissue disease (MCTD). The patients have been followed up for an average of 7 years. The peripheral symptoms on the extremities in particular were exacerbated by exposure to cold and caused much inconvenience and early disability for the patients. In addition to the symptoms generally connected with MCTD, some of the patients presented signs of other diseases of autoimmune type as well. For instance 2 of the patients presented autoimmune thyroiditis and one patient developed myasthenia gravis and cold agglutinin syndrome.
The enlarged intercellular space in the lower malpighian layers of untreated psoriasis contains a fine granular and a coarse granular substance. Both types of this substance increase in amount during 2-6 weeks of retinoid (RO 10-9359) therapy. Retinoid stimulates the formation of these substances, which previously have been termed "mucus". After glutaraldehyde-osmium tetroxide fixation their mucoid nature cannot be ascertained more specific cytochemical techniques being needed for this. A darker, more homogeneous substance in the upper spinous intercellular spaces increases in amount during retinoid therapy. Large amounts of keratinosome-derived lamellae are observed at the boundary of the stratum intermedium and the stratum corneum. Even after 14 weeks of treatment with retinoid or retinoid plus PUVA, at clinical remission, the histological and electron microscopical pictures still show some psoriatic changes, i.e. enlarged intercellular spaces with fine or coarse granular substance.
2 patients with mycosis fungoides at the plaque stage were treated with oral methoxalen and UVA irradiation. 3 months later both developed the skin tumours with highly malignant histological picture. In 1 patient at the tumour stage the skin tumours were spreading during the PUVA treatment. 3 patients at the first or eczematoid stage cleared completely or partially by PUVA treatment.
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