Biomedical subjects
Publications and source records attributed to G Kolde.
The pigmented spindle cell tumor first described by Reed et al. is a benign melanocytic lesion of the skin that frequently remains unrecognized. The tumor is a heavily pigmented macule or plaque that usually appears on the extremities of young patients. The clinical and histological features and the differential diagnosis of pigmented spindle-cell tumor are described with reference to two clinical cases.
Cutis marmorata teleangiectatica congenita is a rare birth defect of unknown etiology. The disease is characterized by reticular teleangiectasias and phlebectasias. Among the associated anomalies, hypoplasia of an affected extremity is most frequently observed. On the basis of two cases, the clinical features of cutis marmorata teleangiectatica congenita are described and the differential diagnosis discussed.
We report on a rare type of leucoplakia of the lip and the prepuce showing the morphological features of epidermolytic hyperkeratosis. The development of squamous cell carcinoma in one of the patients strongly suggests that epidermolytic leucoplakia may represent a precancerous disorder of the transitional epidermis.
The ultrastructural demonstration of Birbeck granules is the most exact morphological marker for Langerhans' cells (LCs). We have developed a specific ultrastructural morphometric technique that enables quantitative evaluation of the numerical and volume density of LCs as well as quantification of cellular details. Evaluation of normal human skin of the hand and lower buttock showed that the epidermis of the hand contained a significantly higher numerical and volume density of LCs. At the cellular level, these LCs exhibited signs of increased metabolic activity, as reflected by the morphometric data of the nucleus, mitochondria, Golgi area, and Birbeck granules. The present technique thus allows functional characterization of LCs at the morphological level.
We report the second family of ichthyosis bullosa, an entity that was first described by Siemens in 1937 and since then has fallen into oblivion. Clinically, ichthyosis bullosa is characterized by blistering resembling epidermolysis bullosa simplex and by generalized, yet circumscribed dark gray hyperkeratoses covering mainly the arms and the legs. Lichenification and superficially denuded areas (mauserung) are further prominent features. Histology disclosed intracorneal blister formation corresponding to the mauserung phenomenon and epidermolytic hyperkeratosis that was confined to the granular layer and to the uppermost layers of the prickle cells. On electron microscopic examination the keratinocytes of these layers displayed structural alterations of tonofilaments as usually observed in epidermolytic hyperkeratosis. Thus ichthyosis bullosa shares with bullous ichthyosiform erythroderma blistering and epidermolytic hyperkeratosis, but can be distinguished from this wellknown disease by the lack of erythroderma, by the mauserung phenomenon, by the confinement of acanthokeratolysis to the superficial layers of the epidermis, and by intracorneal blistering.
An adult patient with multiple unusual histiocytic tumors of the skin is described. As shown by immunohistologic study, electron microscopy, and immunoelectron microscopy, the tumors represent circumscribed proliferations of the Langerhans cell-related indeterminate dendritic cells of the skin. This distinct cutaneous histiocytosis may represent a paraneoplastic syndrome.
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Recently, autosomal-dominant lamellar ichthyosis (ADLI) has been shown to be a new genetic trait with clinical and histologic features similar to those of autosomal-recessive lamellar ichthyosis. In two patients affected with ADLI, the malpighian keratinocytes showed ultrastructural signs of increased cellular metabolism. The tonofilaments and keratohyaline granules were regular in structure and number. However, as a distinctive ultrastructural feature, a prominent transforming zone was found between the granular and horny layers. Moreover, a normal keratin pattern and only a limited number of lipid inclusions were observed in the stratum corneum. Thus, ADLI can be distinguished from the autosomal-recessive forms of lamellar ichthyosis, permitting a correct diagnosis when genetic counselling has to be given in sporadic cases.
Ten patients with moderate to very severe urticaria pigmentosa were studied for the therapeutic effect of photochemotherapy (PUVA; six adults) and selective ultraviolet phototherapy (SUP; four adolescents). Despite a high mean PUVA dosage (138.6 +/- 63.4 J/cm2), only two patients had a very good response, while three had a good response and one had a fair response. On the reduction of the frequency of treatments, the symptoms gradually recurred, and several months after the discontinuation of therapy, the clinical status had reached the level prior to PUVA. The results with SUP were even less encouraging. A number of biophysical and biochemical parameters of the skin were studied in five patients before PUVA treatment, immediately after several months of PUVA treatment and again 5 months after the discontinuation of PUVA treatment. Weal and erythema reactions to intracutaneous skin tests remained unchanged after PUVA, while wealing with topically applied dimethylsulfoxide (DMSO) decreased. Transepidermal water loss was markedly reduced over DMSO weals. Histamine levels, which were elevated in lesional but not in normal skin, dropped with PUVA treatment, but after the discontinuation of treatment, they increased again in the lesions. On reverse-phase high-performance liquid chromatography, two main chemotactic factors, leukotriene B4 and 5-HETE, were identified in lesional skin. Chemotactic activity was elevated in both lesional and uninvolved patient skin, reached normal levels at both sites after PUVA and maintained these low levels for several months after the discontinuation of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
In seven patients with delayed pressure urticaria, the dermal inflammatory infiltrate was analyzed in early (4-6 hours) and late (20-24 hours) wheals by light and electron microscopy and by histomorphometric and immunohistologic methods. Results were compared with findings in normal skin of patients and in wheals induced with concanavalin A and compound 48/80 injections in patients and five healthy volunteers. Pressure wheals were characterized by a mild mononuclear perivascular infiltrate and by patchy dermal infiltrates of eosinophils. Neutrophils were almost completely absent. The number of dermal mast cells was markedly increased in early wheals, whereas their number was decreased in late wheals. Numerous mast cells were degranulated in late wheals, as shown by electron microscopy. On immunohistologic analysis, the perivascular infiltrate consisted primarily of lymphocytes with the helper phenotype. Epidermal OKT6 Ia-positive Langerhans cells were of normal number. The local increase of mast cells, eosinophils, and helper T lymphocytes suggests that pressure wheals result from a cellular immune response to as-yet-unknown antigens that are generated at pressure sites.
A 5-year-old girl with a unilateral linear systematized skin lesion showing the clinical, histological and ultrastructural features of Hailey-Hailey disease is described. We suggest that this relapsing linear acantholytic dermatosis represents a new distinct entity within the spectrum of unilateral linear skin diseases.
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The mononuclear cell infiltrate of 90 superficial spreading melanomas (SSM) of less than or equal to 1.5 mm thickness was analyzed at different locations around and within the tumors. All patients had been followed for at least 5 years, and 20 (22%) had developed metastases. A correlation between the overall inflammatory response in the histologic sections and the prognosis was not observed, nor could a significant distinction be made between metastasizing and nonmetastasizing melanomas when the mononuclear cell infiltrate was analyzed at different locations. A significant clustering of metastasizing tumors was, however, observed in the subgroup with a relatively strong infiltrate within the tumor compared with the overall infiltrate. From this observation, a semiquantitative description of the pattern of the infiltrate was established. It provides an additional parameter for prognostication in flat SSMs and it raises new questions about the role of the local host-tumor interaction.
In six patients with urticaria pigmentosa, population density and ultrastructure of the cutaneous mast cells and histamine levels of the lesional skin were studied before, immediately after, and again 5-8 months after photochemotherapy (PUVA). Immediately after PUVA, the total mast cell number was not reduced, but on separate analysis of the intradermal distribution, significantly fewer mast cells were found in the papillary dermis and correspondingly more mast cells in the adjacent upper dermis. On electron microscopic examination, 4% of the mast cell granules were immature before and 27% after PUVA therapy, based on the lower electron density of the granular matrix. This was associated with a markedly lower histamine content of the lesional skin. Five to eight months after recovery from PUVA, the morphologic changes and the histamine levels had all returned to the pre-PUVA status. These findings were paralleled by a reversal of all clinical beneficial effects that had been observed with PUVA.
We report two cases with ichthyosis vulgaris, hypogenitalism and hypogonadism. So far, little endocrinological information has been available on this association and the exact type of ichthyosis was unknown. Our first patient suffered from very severe hypergonadotropic hypogonadism, whereas the second patient showed normal levels of luteinizing hormone, but slightly elevated follicle stimulating hormone values. In lipoprotein electrophoresis we found fast moving beta-lipoproteins in the first patient and a normal electrophoretic mobility of pre- beta and beta-lipoproteins in the second patient. Correspondingly, steroid sulfatase (STS) testing revealed STS deficiency in the first patient and normal STS activity in the second patient, thus excluding X-linked recessive ichthyosis. These two different types in the association of ichthyosis with hypogenitalism and hypogonadism could not be discriminated by clinical, morphological and cytogenetic studies.
Lamellar ichthyosis (nonbullous congenital ichthyosis) has been explained as an autosomal recessive trait. We have found an autosomal dominant type of this disorder. Four patients, belonging to three consecutive generations of a family, were affected from birth. The disorder was characterized by large, dark brown scales covering the entire body including flexural folds, palms and soles. X-linked recessive ichthyosis was excluded by clinical appearance, pattern of transmission and normal electrophoretic mobility of beta-lipoproteins. Autosomal dominant ichthyosis vulgaris and bullous ichthyosiform erythroderma were excluded by the histological and ultrastructural features. In the absence of a positive family history, this skin disorder would have been taken for autosomal recessive lamellar ichthyosis. This new autosomal dominant type of ichthyosis should be considered for differential diagnosis, when genetic counselling is given in a sporadic case of lamellar ichthyosis.
The ichthyotic skin in X-linked dominant chondrodysplasia punctata was investigated in a four-week-old baby and a fourteen-year-old girl. Histologically, the ichthyosiform erythroderma of the newborn and the ichthyosis of the older child presented as a retention hyperkeratosis with several distinctive features such as calcification of the keratotic follicular plugs, atrophy of the hair follicles and focal hyperpigmentation of the basal keratinocytes. On ultrastructural examination, small to medium sized vacuoles were regularly seen in the thinned granular layer. Some of these vacuoles contained needle-like calcium inclusions. The histologic and ultrastructural findings are therefore characteristic for this rare type of ichthyosis.
Highly purified subfractions of human peripheral blood monocytes, when cultured in the presence of 30% L cell supernatant and 30% horse serum, assumed all the characteristics that define human connective tissue mast cells. After three weeks of culture, 75% of the cells developed metachromasia and granular chloroacetate esterase staining, and their intracellular histamine levels increased from 0.0 to 50.5 ng/10(6) cells. On electron microscopy, the cells developed intracytoplasmic granules with all the features typical for mature and immature mast cells. Cultured cells bound 55 pg 125I-IgE/10(6) cells, while labelling was negligible with cells prior to culture and with heat-denatured 125I-IgE. Fluorescent staining with anti-IgE increased slightly as well, while staining with monoclonal anti-monocyte and anti-HLA-Dr markers decreased. Purified lymphocytes did not assume mast cell characteristics, and lymphokines did not induce or enhance in vitro mast cell development or IgE binding. The data therefore further support the concept that connective tissue mast cells arise from the monocytoid lineage.