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Biomedical subjects

T Salamon

Publications and source records attributed to T Salamon.

At least 19 recordsLinked to original sources
PubMed1993

Regression and disappearance of clinical symptoms in some cases of genodermatoses.

Genetic skin diseases are usually thought of as stable phenotypes caused by a mutant gene. However, a review of the literature on genodermatoses and my personal experience with a number of these disorders show that the concept of a stable phenotype is not always true. A number of genodermatoses actually show a regression of symptoms. Among these are monilethrix, several types of erythrokeratoderma, several forms of palmoplantar keratoderma such as the varians Wachters type and the Richner-Hanhart syndrome, and some forms of epidermolysis bullosa, for example the Dowling-Meara type. Three different reasons possibly underlying the regression of symptoms in genetic diseases are briefly discussed: (1) environmental factors, (2) hormonal influences and (3) movable, transposable elements causing instability of the genome.

Dermatitis, Exfoliative↗
PubMed1991

[Palmoplantar epidermal atrophy with hypokeratosis, dys- and hypotrichosis, hypodontia, enamel and dentin hypoplasia, isolated cleft palate with cleft uvula, strabismus cryptorchism and other anomalies--an undescribed ecto-mesodermal dysplasia?].

A 15-year-old boy had suffered burning of both palms and all fingers during his 2nd year of life. Epitheliazation of the lesions did not occur. In his 6th year, erythema, desquamation and erosion-like alterations appeared spontaneously on the soles of both feet and on the plantar sides of the toes. These alterations were similar to those on the hands. Histologically the lesion on the right sole was covered with a very thin horny layer and thin epidermis. The cells of the epidermis had clear cytoplasm and probably derived from the ductal epithelium of the sweat glands. Under the atrophic epidermis, granulation tissue consisting of lymphocytes, plasma cells, histiocytes, and a few polynuclear cells and eosinophils was seen. The following symptoms were still present in the boy: dys- and hypotrichosis, oligodontia, enamel and dentin hypoplasia, cleft palate and uvula, concomitants strabismus (left eye), and cryptorchidism. Transplantation of the patient's skin from the right thigh to the lesion on the right sole was tried. The authors postulate a previously unreported form of ecto-mesodermal dysplasia in this case.

Abnormalities, Multiple↗
PubMed1989

[The heritability of vitiligo].

Familial cases of vitiligo in 13 families observed by the authors as well as familial cases from the literature were analyzed. The results are presented in four tables. Genetic factors doubtless play a role in the aetiology of this pigmentation anomaly, but the mode of inheritance does not appear to be uniform. This condition seems to be heterogeneous.

Chromosome Aberrations↗
PubMed1988

[4 cases of Richner-Hanhart syndrome (tyrosinemia type II) with neurological symptomatology in a Yugoslav family].

Findings are described in four cases of Richner-Hanhart syndrome in a Yugoslavian family; this disorder is transmitted as an autosomal recessive trait. This family was first reported in 1963. Now tyrosinemia and tyrosinuria can be found in all cases, and neurological symptoms are also present in all cases, whereas earlier only one of them had these symptoms. In the meantime, the lesions on the soles of the feet have been totally excised in three cases, partially in one, and split thickness grafts from the normal skin have been applied. The palmar lesions were treated in the same manner in two cases. The possibilities for surgical treatment of this very painful, inherited form of dermatosis and their consequences are discussed. Surgical treatment is recommended for palmar lesions, but not for the plantar form. The pathogenesis of the dermatologic manifestations in this rare metabolic disease is briefly discussed.

Adult↗
PubMed1987

[Epidermodysplasia verruciformis-like genodermatosis with changes in the nails].

Nine cases of genodermatosis are described, in which the clinical symptoms and light-microscope findings resembled those in epidermodysplasia verruciformis (Lewandowsky-Lutz disease). All had alterations of the fingernails and toenails. Viruses were not found in a excised skin lesions or in the fingernail clipping from the patients. The mode of inheritance of the dermatosis seems to be X-chromosomal and dominant. It is the opinion of the authors that this dermatosis is separate entity from epidermodysplasia verruciformis.

Adolescent↗
PubMed1985

[A case of erythrokeratodermia variabilis].

A case of erythrokeratodermia variabilis in a 7.5-years-old girl with transient achromic lesions and nail changes is described. Histologically peculiar eosinophilic formations with nuclear remnants in the stratum corneum were found. The mother and the grandfather only had hyperkeratotic lesions on the soles.

Child↗
PubMed1984

Electron microscopic study of fingernails in the disease of Mljet (Mal de Meleda).

Results are presented of the electron-microscopic examinations of fingernail clippings from 4 patients suffering from Mljet (Mal de Meleda) disease, of 3 obligate heterozygotes of this autosomal recessively inherited disease, as well as of 3 controls. In the nails of the diseased persons, significantly more osmiophilic material was found than in those of controls. In the nails of heterozygotes, the findings were intermediate between those of patients and controls. In the nails of aged persons, a strongly osmiophilic matrix keratin was found. Histochemically, lipids were demonstrated in the ventral plate of diseased nails. The probable significance and implications of these findings are discussed.

Adult↗